ft.TLS, 

190°  chestnut -;t;ieet, 
philadelphia,  pa. 


Colum&ta  Winibtv&itv    vDVS 
fnttieCitpofiSeto|9orfe 

College  of  ^fjpgtctans  anb  gmrgeong 


Reference  library 


DISEASES 

OF  THE 

NERVOUS  SYSTEM 

GORDON 


DISEASES  OF  THE 

NERVOUS  SYSTEM 


FOR  THE  GENERAL  PRACTITIONER 
AND  STUDENT 


BY 

ALFRED  GORDON,  A.M.,  M.D.   (Paris) 

LATE   ASSOCIATE   IN  NERVOUS   AND   MENTAL   DISEASES,   JEFFERSON  MEDICAL  COLLEGE;   LATE   EXAMINER   OF 
THE  INSANE,  PHILADELPHIA  GENERAL  HOSPITAL;  NEUROLOGIST  TO  MOUNT  SINAI  HOSPITAL,  TO  NORTH- 
WESTERN   GENERAL    HOSPITAL    AND  TO  THE  DOUGLASS  MEMORIAL  HOSPITAL;  MEMBER  OF  THE 
AMERICAN  NEUROLOGICAL  ASSOCIATION;  FELLOW  OF  THE  COLLEGE  OF  PHYSICIANS  OF 
PHILADELPHIA;  CORRESPONDING  MEMBER  OF  SOCIETE  MEDICO-PSYCHOLOGIQUE 
DE    PARIS,    FRANCE;    MEMBER    OF    THE   AMERICAN    INSTITUTE     OF 
CRIMINAL  LAW  AND  CRIMINOLOGY,   ETC. 


SECOND  EDITION,  REVISED  AND  ENLARGED 
WITH  ONE  HUNDRED  AND  SIXTY-NINE  ILLUSTRATIONS 


PHILADELPHIA 
P.    BLAKISTON'S   SON   &   CO. 

1012   WALNUT   STREET 
1913 


Copyright,  1913,  by  P.  Blakiston's  Son  &  Co. 


PREFACE  TO  THE  SECOND  EDITION 

In  presenting  trie  second  edition  of  this  work  I  had  again  in  view 
principally  the  general  practitioner.  The  new  facts  which  have  been 
developed  since  the  first  edition  I  have  endeavored  to  describe  in  a  concise 
but  at  the  same  time  complete  manner.  Each  chapter  almost  without 
exception  has  been  enlarged  and  among  a  number  of  additions  the  fol- 
lowing important  articles  may  be  mentioned:  (i)  Fracture  of  the  Skull; 
(2)  Concussion  of  the  Brain;  (3)  Lumbar  Puncture;  (4)  Cerebro-spinal 
Fluid;  (5)  Wasserman  Reaction;  (6)  Radiculitis;  (7)  Psychoanalysis. 

Treatment  has  received  special  attention  in  accordance  with  the  new 
data  accumulated,  such  as  administration  of  antimeningo coccus  serum, 
of  salvarsan,  surgical  procedures,  etc. 

Alfred  Gordon. 

1812  Spruce  St.,  Philadelphia. 


Digitized  by  the  Internet  Archive 

in  2010  with  funding  from 

Open  Knowledge  Commons 


http://www.archive.org/details/diseasesofnervouOOgord 


PREFACE  TO  THE  FIRST  EDITION 

In  preparing  this  book  for  the  medical  public  I  had  in  view  chiefly 
the  general  practitioner  and  the  student.  To  both  Neurology  lias  always 
appeared  to  be  a  difficult  and  an  insurmountable  subject  and  many  a 
student  hesitates  to  take  up  its  study  in  earnest.  The  fault  lies  partly  in 
the  text-books  and  treatises  in  which  neurological  subjects  are  discussed 
from  the  standpoint  of  the  expert  neurologist.  In  my  daily  association 
with  students  and  general  practitioners  I  have  invariably  heard  this  con- 
tinuous complaint,  viz.,  that  they  cannot  get  hold  of  a  book  on  Neurology 
which  could  give  them  a  plain  and  practical  account  of  diseases  of  the 
nervous  system.  It  is  the  want  of  such  a  work  that  I  have  endeavored  to 
meet  in  the  modest  volume  I  am  offering. 

The  modern  physician  is  not  satisfied  with  a  mere  enumeration  of  facts. 
In  reading  a  description-  of  any  malady  he  wishes  also  to  know  the  reason 
of  the  disturbed  functions  and  the  anatomical  substratum  of  the  morbid 
phenomena.  Otherwise  speaking  he  feels  that  he  must  know  the  relation 
of  a  certain  manifestation  to  the  normal  and  morbid  physiology  of  an 
affected  tissue  or  organ.  The  knowledge  of  pathology  is  therefore  a  sine 
qua  non  to  every  thinking  man.  This  chapter  must  therefore  precede  any 
other  in  giving  an  account  of  a  certain  disease. 

As  I  am  aiming  almost  exclusively  to  present  Nervous  Diseases  from 
a  practical  standpoint,  I  naturally  avoided  too  technical  and  debatable 
points  of  pathology,  but  on  the  contrary  endeavored  to  present  the  most 
essential  changes  necessary  for  a  thorough  understanding  of  various  clin- 
ical manifestations. 

In  discussing  the  symptomatology  I  point  out,  whenever  it  is  possible,  the 
direct  relation  between  certain  phenomena  and  the  pathological  changes 
so  as  to  give  the  reader  an  intelligent  idea  of  the  morbid  symptoms. 

Each  form  of  functional  or  organic  nervous  disease  is  also  discussed 
from  the  standpoint  of  differential  diagnosis.  All  possible  affections  which 
may  simulate  a  given  disease  have  been  taken  up  seriatim  and  differences 
emphasized. 

The  course  of  the  diseases,  their  mode  of  termination,  their  prognosis 
and  the  etiology  have  been  given  full  consideration.  In  describing  the 
latter,  the  most  well  known  and  well  established  factors  have  been  pointed 
out  first.  The  reader  may  be  surprised  to  find  Etiology  placed  in  some 
chapters  before  Symptomatology  and  in  others  immediately  before  Treat- 

vii 


Vlll  PREFACE 

ment.  This  was  arranged  according  to  the  importance  Etiology  plays  in 
certain  diseases  or  according  to  the  amount  of  knowledge  we  possess  of  the 
causative  factors  in  various  diseases. 

Considerable  space  has  been  devoted  to  Treatment.  Only  the  most 
useful  and  the  best  known  devices,  appliances,  operations  and  drugs  are 
described.  Medications  that  are  uncertain  as  to  their  therapeutic  value 
are  omitted  or  else  only  mentioned. 

True  to  my  original  aim  I  have  endeavored  to  present  to  the  reader 
only  the  most  essential  points,  whether  it  was  in  Pathology,  Symptomatol- 
ogy, Pathogenesis,  Etiology  or  Treatment.  The  latest  views,  ideas  and 
thoughts  have  been  presented  as  far  as  it  was  possible.  Intentionally  I 
avoided  details  on  disputable  questions  and  omitted  them  altogether  when- 
ever I  could  without  sacrificing  the  clearness  of  the  subject. 

A  Chapter  on  the  Method  for  examination  of  patients  precedes  the 
description  of  diseases  of  then  ervous  system.  In  it  are  indicated  what 
phenomena  are  considered  normal  or  abnormal.  I  have  described  here 
the  motor,  sensory  and  trophic  phenomena,  also  the  reflexes,  the  state  of 
sphincters  and  electrical  contractility  of  muscles  or  nerves. 

Finally  a  chapter  on  the  Normal  Anatomy  of  Brain  and  Cord,  also 
Malformations  of  the  Nervous  System,  has  been  added.  Detailed  de- 
scriptions have  been  omitted  whenever  it  was  possible  and  instead  clear 
illustrations  are  given. 

On  the  whole  I  feel  that  I  am  presenting  a  practical  book  to  the  average 
physician,  but  if  also  the  neurologist,  the  teacher,  the  advanced  student 
may  find  in  it  some  ready  references  which  they  may  peruse  in  their  scien- 
tific studies,  my  labors  will  be  more  than  compensated. 

A  word  of  thanks  is  due  to  the  publishers.  They  have  facilitated  my 
task  by  allowing  a  large  number  of  illustrations,  without  which  no  modern 
scientific  work  can  be  satisfactory. 

Alfred  Gordon. 

1430  Pine  St.,  Philadelphia, 


CONTENTS 

CHAPTER  I 

Anatomy  and  Physiology  of  the  Central  Nervous  System 

Page 

Spinal  Cord i 

Meninges  or  the  Cord 9 

Blood  Supply  of  the  Cord n 

Rhombencephalon " 

Medulla  oblongata " 

Pons 15 

Fourth  ventricle      19 

Mesencephalon  (Middle  Brain)     20 

Area  of  Crura  and  Corpora  quadrigemina 20 

Diencephalon  (Interbrain) 24 

Area  of  Optic  Thalami  and  Third  ventricle 24 

Cerebral  Hemispheres.    Telencephalon 26 

Gray  substance 27 

White  substance 31 

Tracts 32 

Cerebellum 4° 

Meninges  of  the  Brain     43 

Blood  Supply  of  the  Brain 46 

Histological  Elements  of  the  Central  Nervous  System 49 

Neurone  Doctrine  and  Secondary  Degeneration 5° 

Malformations  of  the  Central  Nervous  System 52 

CHAPTER  II 

Method  of  Examination  for  Diagnosis  of  Nervous  Diseases 

I.  Motor  Phenomena 59 

II.  State  of  Nutrition  of  Muscles 62 

III.  Electrical  Contractility 63 

IV.  Sensory  Phenomena 68 

V.  Special  Senses •    •  7° 

VI.  Cranial  Nerves 7* 

VII.  Speech  and  Writing 71 

VIII.  Reflexes 71 

IX.  Sphincters 74 

X.  Vasomotor  and  Trophic  Disturbances 74 

CHAPTER  III 

Cerebral  Localizations 

Motor  Centers 75 

Speech  Centers 78 

Sensory  Centers 79 

ix 


X  CONTENTS 

Page 

Special  Sensations 80 

Intelligence     81 

CHAPTER  IV 

Apoplexy 

Hemorrhage 82 

Embolism 86 

Thrombosis 86 

Hemiplegia 9° 

Delayed  Apoplexy 101 

Intermittent  Closing  of  Cerebral  Arteries  and  Transitory  Hemiplegia  ....   102 

CHAPTER  V 

Encephalitis 

Acute  Non-suppuratwe  Form 104 

Suppurative  Form.     Abscess  of  the  Brain 106 

Chronic  Encephalitis II2 

Infantile  spastic  hemiplegia nS 

Spastic  Diplegia.     Little's  disease 7 i*7 

CHAPTER  VI 

Jacksonian  or  Focal  Epilepsy I24 

Epilepsia  Partialis  Continua 128 

CHAPTER  VII 
Aphasia I3° 

CHAPTER  VIII 
Hemianopsia I4° 

CHAPTER  IX 
Tumors  of  the  Brain I43 

CHAPTER  X 
Hydrocephalus l&2 

CHAPTER  XI 

Diseases  of  the  Basal  Ganglia 
Optic  Thalamus.     Corpora  Striata.     Corpora  Quadrigemina 167 

CHAPTER  XII 
Meningitis I7I 

CHAPTER  XIII 
Thrombosis  of  the  Intracranial  Sinuses J99 

CHAPTER  XIV 

Circulatory  Disturbances  of  the  Brain 

ANiEMiA.     Hyperemia 2°2 


CONTENTS  XI 

Page 
CHAPTER  XV 

Fractures  of  the  Skull '       205 

CHAPTER   XVI 
Concussion  of  the  Brain 209 

CHAPTER  XVII 

Diseases  of  the  Cerebellum 

Tumors 213 

Abscess 218 

Cerebellar  Heredo-ataxia 219 

Hemorrhage  and  Softening 221 

CHAPTER  XVIII 
Diseases  of  the  Medulla,  Pons  and  Fourth  Ventricle 

A.  Acute  Superior  Polioencephalitis 223 

B.  Chronic  Superior  Polioencephalitis 224 

C  Acute  Inferior  Polioencephalitis 226 

D.  Chronic  Inferior  Polioencephalitis 226 

E.  PSEUDO-BULBAR  PALSY 229 

F.  Myasthenia  Gravis 231 

G.  Hemorrhage  and  Softening  of  the  Medulla 234 

H.  Occlusion  of  Posterior  Inferior  Cerebellar  Artery 235 

I.  Compression  of  the  Medulla 235 

Diseases  of  the  Pons 236 

Hemorrhage.     Softening.     Tumors 237 

Crossed  Paralysis 239 

Peduncular  Syndrome 241 

CHAPTER  XIX 

Diseases  of  the  Spinal  Cord 

A.  Systemic  Diseases  of  the  Cord      242 

I.  Tabes 242 

II.  Spastic  Paraplegia 256 

Family  Spastic  Paraplegia 257 

Paraplegia  of  the  aged 258 

III.  x\taxic  Paraplegia      259 

IV.  Friedreich's  Ataxia 261 

V.  Acute  Anterior  Poliomyelitis 264 

VI.   Chronic  Anterior  Poliomyelitis 272 

VII.  Amyotrophic  Lateral  Sclerosis 272 

B    Non-systemic  Diseases  of  the  Cord 272 

I.  Myelitis 272 

II.  Hematomyelia 279 

III.  Divers'  Paralysis 284 

IV.  Syringomyelia 286 

V.  Diseases  of  Conus  Medullaris  and  Cauda  Equina 291 

VI.  Disseminated  Sclerosis 295 


XU  CONTENTS 

Page 

Secondary  Affections  of  the  Spinal  Cord 302 

I.  Traumatic  Lesions  of  the  Cord 302 

Concussion.     Contusion 302 

Sudden  Compression.     Laceration 303 

II.  Slow  Compression.     Tumors.     Pott's  Disease 306 

CHAPTER  XX 

Muscular  Atrophies 314 

I.  Progressive  Muscular  Atrophy  of  Spinal  Origin 314 

Progressive  muscular  atrophy  of  infants 318 

Amyotrophic  Lateral  Sclerosis 319 

II.  Myopathy 321 

III.  Primary  Neurotic  Atrophy      325 

IV.  Arthritic  Muscular  Atrophy 327 

Amyotonia  Congenita 327 

Myotonia  Atrophica 329 

Diseases  of  Spinal  Meninges 329 

Intermittent  Claudication  of  the  Spinal  Cord 334 

CHAPTER  XXI. 
Syphilis  of  the  Nervous  System 336 

CHAPTER  XXII 
Paresis 353 

CHAPTER  XXIII 

Lumbar  Puncture  and  Cerebro-spinal  Fluid.    Wassermann  Reaction 369 

CHAPTER  XXIV 

Diseases  of  the  Peripheral  Nervous  System 

Neuritis 377 

Neuroma 384 

Multiple  Neuritis 385 

A.  Alcoholic  Multiple  Neuritis 387 

B.  Lead  Multiple  Neuritis 389 

C.  Arsenical  Multiple  Neuritis 390 

D.  Diphtheritic  Multiple  Neuritis 390 

E.  Carbonic  Gas  Multiple  Neuritis 391 

F.  Mercurial  Multiple  Neuritis 392 

G.  Puerperal  Multiple  Neuritis 392 

H.  Beriberi  Multiple  Neuritis      392 

I.  Lepra  Multiple  Neuritis 393 

K.  Senile  Neuritis 394 

Acute  Ascending  Paralysis 396 

Periodic  Paralysis      398 

Diseases  of  Individual  Nerves     399 

I.  Paralysis  of  Cranial  Nerves 399 

II.  Paralysis  of  Spinal  Nerves       415 

A.  Upper  Cervical  Nerves 415 

B.  Lower  Cervical  Nerves  (Brachial  Plexus) 416 

C.  Lumbo-sacral  Nerves 425 


CONTENTS  Xlll 

Page 

Radiculitis 429 

Neuralgia  in  General | 430 

Neuralgia  of  Individual  Nerves 435 

Neralgia  Paraesthetica  and  Intermittent  Claudication 445 

Herpes  Zoster      447 

CHAPTER  XXV 

Functional  Nervous  Diseases 

Neurasthenia 451 

psychasthenia 456 

Hypochondria 458 

Anxiety  Neurosis 464 

Hysteria 464 

Epilepsy 482 

Chorea 495 

Athetosis 503 

Dystonia  Musculorum  Deformans 505 

Tic 507 

Facial  Spasm 514 

Myoclonia 516 

Tetany 519 

Myospasm  from  Intense  Heat 524 

Myotonia  Congenita  (Thomsen's  Disease) 525 

Occupation  Neuroses - 527 

Paralysis  Agitans 529 

Akinesia  Algera 533 

Headache 535 

Migraine 539 

Vertigo 544 

CHAPTER  XXVI 

Traumatic  Neuroses  and  Psychoses.     Medico-legal  Considerations 548 

CHAPTER  XXVII 

Diseases  of  the  Sympathetic  System 

Trophoneuroses.     Angioneuroses 

Exophthalmic  Goiter 560 

Myxcedema      567 

Acromegaly 572 

Gigantism 575 

Achondroplasia 576 

Adiposis  Dolorosa 577 

Scleroderma 579 

Facial  Hemiatrophy 581 

Facial  Hemihypertrophy 583 

Acroparesthesia      583 

Angioneurotic  (Edema 584 

Hereditary  (Edema  of  the  Legs 585 

Erythromelalgia 585 

Raynaud's  Disease      587 


XIV  CONTENTS 

Page 
CHAPTER  XXVIII 

Nervous  Symptoms  Produced  by  Intoxications 

A    Metallic  Poisons 591 

I.  Lead  Intoxication      591 

II.  Arsenical  Intoxication 592 

III.  Mercurial  Intoxication 593 

IV.  Carbon  Monoxide  Intoxication 593 

V.  Manganese  Intoxication 594 

B.  Organic  Poisons 595 

I.  Alcoholism      '. 595 

II.  Morphinism 599 

III.   Cocainism 601 

CHAPTER  XXIX 

Nervous  Symptoms  Caused  by  Some  Special  Infections 602 

Tetanus 602 

Hydrophobia 605 

Pellagra 608 

Index 609 


Diseases  of  the  Nervous  System 


CHAPTER   I 


ANATOMY  AND  PHYSIOLOGY  OF  THE  CENTRAL 
NERVOUS  SYSTEM 

SPINAL  CORD 

The  cord  covered,  by  three  membranes  is  placed  in  the  vertebral 
canal  and  extends  from  the  upper  border  of  the  atlas  down  to  the  upper 
border  of  the  second  lumbar  vertebra.  It  occupies  therefore  only  two- 
thirds  of  the  vertebral  canal,  viz.  its  cervical  and  thoracic  portions.  It 
is  approximately  a  cylindrical  body  presenting  two  enlargements  and  a 
conical  termination.  Its  length  is  about  45  cm.  (18  inches)  in  the  male 
and  41  cm.  (16  inches)  in  the  female. 

The  cord  is  continuous  above  with  the  medulla  and  below  it  forms  a 
thread-like  termination  (filum  terminate)  which  extends  to  the  coccyx 
to  which  it  is  attached  (Fig.  1). 

The  spinal  cord  is  divided  into  the  following  segments:  cervical, 
thoracic,  lumbar  and  sacral  or  Conus  Medullaris.  The  Cervical  and  Lum- 
bar segments  are  the  thickest  parts  of  the  cord,  viz.  the  enlargements 
mentioned  above. 

The  segments  correspond  to  the  following  vertebrae.  The  Cervical 
enlargement,  which  supplies  nerves  to  the  upper  extremities  also  gives 
origin  to  the  phrenic  nerve,  extends  from  the  third  cervical  to  the  second 
thoracic  vertebra  and  has  its  maximum  of  development  at  the  level  of 
the  sixth  cervical  vertebra.  The  portion  of  the  cord  above  the  enlarge- 
ment corresponds  to  the  first  two  cervical  vertebrae.  The  Thoracic 
segment  extends  from  the  second  to  the  ninth  thoracic  vertebra.  The 
Lumbar  enlargement,  which  supplies  nerves  to  the  lower  extremities, 
commences  at  the  level  of  the  ninth  thoracic  and  terminates  at  the  lower 
border  of  the  first  lumbar  vertebra.  Its  maximum  corresponds  to  the 
twelfth  thoracic  vertebra. 

The  Conus  Medullaris  or  sacral  segment  is  the  very  small  conical 
portion  extending  from  the  first  to  the  second  lumbar  vertebra. 

Exterior  of  the  Cord.— The  cord  is  divided  into  two  halves  by  an 


THE    CENTRAL    NERVOUS    SYSTEM 


CIA  VA 

FUNICUL  US  CUNEA  TUS 


POSTERO-MEDIAN  SULCUS- 


POSTERO-LA  TERAL 
SULCUS 


POSTERO-LA  TERAL , 

SULCUS 


POSTERO-MEDIA  N  S  ULCUS 


CERVICAL 
EN  LA R GEMENT 


FILUM 
TERMINALE 


LUMBAR 
ENLA R GEMENT 


CONUS 
MEDULLARIS 


OLIVARY  BODY 
LATERAL  FUNICULUS 
DECUSSATION  OF  PYRAMIDS 

ANTERIOR  MEDIAN  FISSURE 


SECTION  OF  MED  ULLA 
OBLONGATA 

||- ANTERO-LATERAL  SULCUS 
{Line  of  ventral  nerve-roots) 


ANTERIOR  MEDIAN  FISSURE 


Fig.  i. — Posterior  and  Anterior  Views  of  the  Spinal  Cord.     {Morris,  modified  from 

Quain.) 


THE  CENTRAL  NERVOUS  SYSTEM 


anterior  and  posterior  median  fissure.  The  anterior  is  a  deep  and  broad 
fissure  and  contains  a  duplication  of  the  pia-mater  with  its  important 
blood  vessels.  The  posterior  median  fissure  is  simply  a  sulcus,  a  septum. 
Each  half  of  the  cord  is  divided  by  two  sulci  into  three  portions.  They 
are:  the  postero-lateral  sulcus,  which  receives  the  posterior  sensory  roots, 
and  the  antero-lateral  sulcus,  which  is  the  place  of  exit  of  the  anterior 
roots. 

The  portions  of  the  cord  between  the  sulci  present  in  each  half  longi- 
tudinal columns,  viz.  posterior,  lateral  and  anterior. 

Interior  of  the  Cord. — A  transverse  section  shows  that  the  spinal 


POSTERIOR  FUNICULUS 


_  •  RETICULAR 
FORMA  TION 


_  LATERAL 
FUNICULUS 


ANTERIOR  WHITE 
COMMISSURE 

ANTERIOR  FUNICULUS 


{After  Morris'  Anatomy. 


cord  consists  of  a  central  gray  and  a  peripheral  white  substance  (Figs.  2, 
3,  4,  5,  6  and  7). 

Gray  Substance. — It  presents  two  symmetrical  halves  united  in  the 
middle  line  by  a  gray  commissure  in  the  center  of  which  is  the  central 
canal.  The  two  halves  with  the  intermediate  commissure  give  the  im- 
pression of  the  letter  H.  The  central  canal  extends  through  the  entire 
length  of  the  cord. 

Each  half  of  H-shaped  gray  mass  has  an  anterior  and  posterior  por- 
tion, called  "cornua."  The  anterior  cornu  is  distinguished  by  its  larger, 
wider  and  thicker  appearance  than  the  posterior.  Besides,  it  is  separated 
from  the  periphery  of  the  cord  by  the  white  substance.  Between  the  an- 
terior portions  of  the  two  anterior  cornua  lies  a  bundle  of  fibers  crossing 
the  middle  line,  which  constitutes  the  anterior  white  commissure. 


THE  CENTRAL  NERVOUS  SYSTEM 


FASCICULUS  CUNEATUS 
I 


FASCICULUS 
GRACILIS 


POSTERIOR  SEPTUM 


-  -DORSAL  ROOT 

,  DORSAL  HORN 


ANTERIOR 
MEDIAN  FISSURE 


VENTRAL  HORN 
-  "  VENTRAL  ROOT 


Fig.  3. — Cervical .VI.     (After  Morris'  Anatomy.) 


Fig.  4.— Thoracic  VIII.     (After  Morris'  Anatomy.) 


THE    CENTRAL    NERVOUS    SYSTEM 


Fig.  5. — Lumbar  III.     (After  Morris'  Anatomy.) 


Fig.  6. — Sacral  IV.     (After  Morris'  Anatomy.) 


Fig.  7.— Coccygeal.     (After  Morris'  Anatomy.) 

Figs.  2  to  7. — Transverse  Sections  prom  Different  Segments  of  the  Spinal  Cord, 
showing  Shape  and  Relative  Proportions  of  Gray  and  White  Substance  in  the  Dif- 
ferent Segments  Represented.     (Morris'  Anatomy.) 


6  THE  CENTRAL  NERVOUS  SYSTEM 

The  posterior  cornu  is,  thin,  long  and  extends  to  the  periphery  of  the 
cord.  It  presents  a  head,  neck  and  base.  The  head  is  surrounded  by 
the  Gelatinous  substance  of  Rolando.  The  latter  is  particularly.marked 
in  the  cervical  region. 

In  the  upper  thoracic  segment  of  the  cord  there  is  a  lateral  prominence 
of  gray  matter  situated  between  the  bases  of  the  anterior  and  posterior 
cornua.  This  is  the  so-called  lateral  cornu.  It  is  not  found  in  the  cervi- 
cal or  lumbar  region. 

The  anterior  and  posterior  cornua  retain  their  relative  size  through 
the  entire  cord,  but  the  amount  of  gray  matter  in  them  varies  according 
to  the  level  of  the  cord.  Thus  in  the  lumbar  and  cervical  enlargements 
they  reach  the  maximum.  In  the  midthoracic  region  they  are  at  the 
minimum. 

The  gray  matter  is  composed  essentially  of  cells.  The  latter  are 
arranged  in  groups  in  each  cornu.  In  the  anterior  cornu  there  are  mainly: 
an  external  group  which  is  the  principal  origin  of  the  anterior  roots  and 
an  internal  group  which  sends  out  fibers  for  formation  of  the  white  com- 
missure (see  above).  A  special  group  of  cells  (vesicular  column  of  Clarke) 
is  situated  in  the  internal  portion  of  the  base  of  the  posterior  cornu. 
It  extends  from  the  eighth  cervical  to  the  third  lumbar  segment  of  the 
cord.  The  majority  of  these  cells  are  the  origin  of  the  direct  cerebellar 
tract  of  the  same  side  (see  below). 

The  gelatinous  substance  of  Rolando  is  rich  in  cells. 

White  Substance. — The  fibers  composing  it  originate  from  the  cells 
of  the  gray  matter  of  the  cord  and  from  the  cells  of  the  brain  and  cere- 
bellum. They  form  systems  of  fibers  or  columns  with  different  physio- 
logical functions  (Fig.  8). 

I.  Posterior  Columns. — Uniform  in  the  lower  half  of  the  cord,  they 
are  divided  into  two  distinct  columns  at  the  level  of  the  upper  thoracic 
and  cervical  segments.  The  median  bundle  is  the  column  of  Goll  and  the 
outer  bundle  is  the  column  of  Burdach.  They  are  composed  essentially 
of  fibers  of  ramification  of  the  posterior  roots. 

The  fibers  of  the  posterior  roots  emanate  mostly  from  the  cells  of  the 
spinal  ganglia.  The  axone  leaving  its  cell  undergoes  a  division  in  the 
shape  of  letter  T.  One  branch  goes  to  the  periphery  and  the  other  enters 
the  posterior  root.  The  fibers  of  the  latter  ascend  and  subdivide  in  as- 
cending and  descending  branches.  The  descending  ones  are  mostly  short 
fibers,  give  off  collaterals  and  enter  the  cells  of  the  gray  matter.  The 
ascending  branches  are  long  and  short.  The  first  extend  to  the  medulla 
where  they  terminate  in  the  nuclei  of  Goll  and  Burdach.  The  latter 
end  in  the  cells  of  the  gray  matter. 


THE  CENTRAL  NERVOUS  SYSTEM  7 

The  root  fibers,  upon  their  entrance  into  the  cord,  are  divided  into 
two  groups:  an  external,  which  constitutes  Lissauer's  tract  or  marginal 
zone  of  Lissauer  (in  formation  of  which  participate  also  axones  from 
the  posterior  horns)  and  an  internal  which  constitutes  the  posterior 
columns  properly. 

Both  branches  of  each  root-fiber  give  off  collaterals.  They  are  short 
and  long.     The  short  ones  end  around  the  cells  of  the  posterior  cornua 


VIII 


Fig.  8. — Schematic  Representation  of  the  Situation  or  the  Various  Tracts  of  Fibers 
in  the  Spinal  Cord.  {Gordinier.) 
I.  Direct  pyramidal  tract.  II.  Descending  tract  of  Marchi  and  Lowenthal.  III.  Olivary 
or  triangular  tract.  IV.  Antero-lateral  ground  bundles  of  fibers.  V.  Antero-lateral  ascending 
tract  of  Gowers.  VI.  Lateral  limiting  layer.  VII.  Direct  cerebellar  tract.  VIII.  Crossed 
pyramidal  tract.  IX.  Lissauer's  tract.  X.  Middle  root  zone.  XI.  Posterior  root  zone.  XII. 
Postero-internal  or  column  of  Goll.  XIII.  Sep  to-marginal  tract.  XIV.  Comma  tract  of 
Schultze.     XV.  Anterior  root  zone.     XVI.  Cornu-commissural  tract. 

and  of  the  cells  of  Clarke;  they  also  cross  the  gray  commissure  and  end 
in  the  cells  of  the  posterior  cornua  of  the  opposite  side. 

The  long  collaterals  end  around  the  cells  of  the  anterior  cornua.  They 
therefore  carry  to  the  motor  cells  of  the  anterior  cornua  peripheral  sensory 
impulses,  received  from  the  periphery  through  the  spinal  ganglia.  They 
therefore  form  a  part  of  the  arc  reflex. 

In  the  posterior  columns  there  are  also  fibers  originating  in  the  cord 
itself  (endogenous).  In  the  very  anterior  portion  behind  the  gray  com- 
missure there  is  the  Cornu-cornmissural  tract.  Its  fibers  originate  in 
the  cells  of  the  posterior  cornu.     They  have  an  ascending  direction. 


8  THE  CENTRAL  NERVOUS  SYSTEM 

In  the  cervical  region  there  is  a  small  bundle  situated  in  the  middle  of 
the  posterior  columns  and  called  comma  of  Shultze.  A  small  bundle 
situated  in  the  thoracic  region  at  the  periphery  (Hoche's  bundle),  in  the 
lumbar  region  on  each  side  of  the  median  septum  and  in  the  middle  (Flech- 
sig's  oval  bundle)  and  in  the  sacral  region  on  the  posterior  and  internal 
portion  (Gombault's  and  Philippe's  triangle)  are  probably  the  same  system 
of  fibers.     All  these  separate  fasciculi  have  a  descending  course. 

The  posterior  columns  proper  are  composed  of  sensory  neurones 
conducting  sensations  from  the  periphery  to  the  cerebral  cortex  and  have 
therefore  an  ascending  course. 

II.  Pyramidal  Bundle.  Motor  Pathway. — Originating  in  the  motor 
area  of  the  brain  it  descends  toward  the  base  through  the  internal  cap- 
sule lower  down  through  the  pons  and  in  the  medulla  forms  the  pyramids. 
In  the  lower  part  of  the  medulla  the  largest  majority  of  the  pyramidal 
fibers  decussate  with  those  of  the  opposite  side. 

The  non-decussating  fibers  (Tiirck's  bundle)  descend  in  the  anterior 
columns  of  the  cord  occupying  the  portion  near  the  median  fissure.  It 
is  called  "direct  pyramidal  tract."  It  extends  down  to  the  middle  thor- 
acic segment  of  the  cord. 

The  decussating  fibers  (crossed  pyramidal  tract)  after  passing  from 
the  medulla  through  the  anterior  cornua  on  the  opposite  side  of  the  cervi- 
cal cord  (first  and  second  segments)  are  placed  very  posteriorly  in  the  lat- 
eral portion  of  the  cord  through  its  entire  length.  It  is  separated  from 
the  periphery  of  the  cord  by  the  direct  cerebellar  tract,  except  in  the  lum- 
bar region  where  the  latter  does  not  exist. 

The  pyramidal  fibers  with  their  collaterals  terminate  around  the 
cells  of  the  anterior  cornua. 

They  are  motor  and  centrifugal  (descending);  they  transmit  to  the 
motor  cells  of  the  anterior  cornua  voluntary  impulses  from  the  cortical 
motor  centers. 

III.  Direct  Cerebellar  Tract  (Flechsig). — The  fibers  originate  in  the 
cells  of  Clarke's  columns  and  commence  at  the  level  of  the  first  lumbar 
segment.  They  occupy  the  periphery  of  the  postero-lateral  portion  of 
the  cord. 

They  are  sensory  and  centripetal,  have  an  ascending  course  and  at  the 
level  of  the  medulla  enter  the  restiform  bodies  (inferior  cerebellar  pe- 
duncle) to  terminate  in  the  cerebellum.  They  carry  to  the  cortical  cells 
of  the  latter  impressions  received  by  Clarke's  cells  from  the  posterior  roots. 

IV.  Gowers'  Tract  (Antero-lateral  fasciculus). — The  majority  of  its 
fibers  originate  from  cells  of  the  anterior  cornua  of  the  opposite  side,  al- 
though the  exact  source  is  not  known.     The  bundle  is  situated  antero- 


THE  CENTRAL  NERVOUS  SYSTEM  9 

laterally,  in  front  of  the  crossed  pyramidal  and  in  front  of  and  internally 
to  the  direct  cerebellar  tract.  It  commences  at  the  level  of  the  dorso- 
lumbar  region. 

Its  fibers  are  sensory  and  have  an  ascending  course.  Some  of  its 
fibers  go  to  the  cerebellum  through  the  superior  cerebellar  peduncle  and 
terminate  in  the  cortex  of  the  superior  vermis.  Others  enter  the  restiform 
body.     A  few  reach  the  anterior  corpora  quadrigemina. 

V.  Antero-lateral  Ground  Bundle  surrounds  immediately  the  antero- 
lateral portion  of  the  gray  matter.  It  reaches  the  periphery  only  in  front 
of  the  anterior  cornu.  Its  fibers  originate  partly  in  the  cells  of  the  gray 
matter  and  serve  for  associating  various  levels  of  the  cord.  It  contains 
also  fibers  descending  from  the  cerebellum,  red  nucleus,  Deiter's  nucleus, 
corpora  quadrigemina  and  optic  thalamus.  Loewenthal's  bundle  or 
anterior  marginal  fasciculus  is  a  narrow  band  occupying  the  border  of  the 
cord  between  the  anterior  end  of  Gower's  tract  and  the  anterior  median 
fissure.  It  originates  in  the  roof  nucleus  of  the  cerebellum  (nucleus 
fastigii)  and  terminates  about  the  cells  of  the  anterior  horns.  Its  fibers 
are  therefore  descending. 

Roots  of  the  Spinal  Nerves. — The  axones  emanating  from  the  cells  of 
the  anterior  cornua,  reach  the  periphery  of  the  cord  and  form  the  anterior 
roots.  After  piercing  the  membranes  of  the  cord  they  advance  to  the 
intervertebral  foramina  and  beyond  the  spinal  ganglia  situated  in  those 
foramina  they  join  the  posterior  roots  to  form  a  spinal  nerve. 

The  function  of  the  anterior  roots  is  motor  (and  trophic). 

The  posterior  roots  are  formed  of  sensory  fasciculi  coming  from  the 
periphery,  penetrate  the  intervertebral  foramina  where  they  meet  the 
spinal  ganglia.  From  there,  covered  by  the  three  membranes  of  the  cord, 
they  reach  the  postero-lateral  sulcus  and  enter  the  cord. 

The  spinal  nerves  formed  of  the  junction  of  the  anterior  and  posterior 
roots  are  31  pairs  in  number. 

The  roots  of  the  first  cervical  nerve  are  horizontal.  Beginning  with 
the  thoracic  nerve  the  direction  of  the  roots  is  very  oblique,  so  that  the 
last  roots  extend  through  a  distance  of  several  vertebras. 

MENINGES  OF  THE  SPINAL  CORD 

The  cord  is  surrounded  by  three  membranes:  dura -mater,  arachnoid 
and  pia-mater.     (Fig.  9.) 

Dura  consists  of  one  layer.  It  is  a  resistant  membrane  and  contrary 
to  the  dura  of  the  brain,  does  not  constitute  the  internal  periosteum  of 
the  spinal  canal.  It  is  separated  from  the  bony  walls  of  the  spinal  canal 
by  a  loose  areolar  tissue  with  a  plexus  of  veins. 


IO 


THE  CENTRAL  NERVOUS  SYSTEM 


It  commences  at  the  foramen  magnum  and  terminates  at  the  level  of 
the  third  piece  of  the  sacrum,  while  the  spinal  cord  ends  only  at  the  level 
of  the  second  lumbar  vertebra.  Below  the  cord  the  cavity  of  the  dural 
sac  is  occupied  by  a  bundle  of  nerves — cauda  equina — in  the  midst  of 
which  is  seen  the  filum  terminale.  Below  the  third  sacral  vertebra  the 
dura  becomes  only  a  filament  extending  to  the  coccyx  (coccygeal  ligament). 
It  is  attached  to  the  canal  by  ligaments  and  prolongations  which  together 
with  the  pia  and  arachnoid  accompany  and  surround  the  spinal  roots  on 
their  way  to  the  intervertebral  foramina. 


Ijigamentum  dentieulat um 


INTERVERTEBRAL 
FORAMEN 


BODY  OF  VERTEBRA 
Periosteum 

Dura  mater 

SUBDURAL  CAVITY 
Arachnoid 

SUBARACHNOID 

CA  VITY 
Pia  mater 


Fig.  9. — Diagram  showing  Relations  of  Meninges  to  Spinal  Nerve-roots.     (Morris' 

Anatomy.) 


The  inner  surface  of  the  dura  is  attached  to  the  pia-mater  by  dentate 
ligaments  through  the  arachnoid.  Between  the  dura  and  the  arachnoid 
there  is  the  so-called  subdural  cavity  which  contains  cerebro-spinal  fluid. 

Arachnoid. — It  covers  the  cord  and  the  cauda  equina  and  is  continu- 
ous with  the  arachnoid  of  the  brain.  Between  it  and  the  pia  there  is 
the  so-called  sub-arachnoid  space  containing  a  reticulum  the  meshes  of 
which  are  occupied  by  the  cerebro-spinal  fluid.  The  above  mentioned 
dentate  ligaments  and  the  septum  posticum  keep  it  attached  to  the  pia 
and  dura.     It  gives  off  prolongations  to  the  spinal  roots. 

Pia. — It  is  the  most  internal  of  the  three  meninges.  It  is  an  extremely 
vascular  membrane  closely  adherent  to  the  cord.  It  is  thicker  in  the 
spinal  cord  than  in  the  brain.     It  is  composed  of  two  layers.     It  forms  a 


THE  CENTRAL  NERVOUS  SYSTEM  II 

fold  in  the  anterior  fissure  of  the  cord.  It  sends  off  prolongations  to  the 
spinal  roots.  At  the  level  of  the  filum  terminale  it  behaves  like  the  dura 
(see  above).  Ligamentum  dentate  mentioned  above  is  a  fold  of  the  pia 
and  presents  processes  which  are  attached  on  the  inner  surface  of  the  dura 
between  the  roots  of  the  spinal  nerves. 

BLOOD  SUPPLY  OF  THE  SPINAL  CORD 

The  main  arteries  of  the  spinal  cord  are  three  in  number,  viz.  one 
anterior  spinal  artery  along  the  anterior  spinal  fissure,  two  posterior 
spinal  arteries,  one  on  each  side  behind  the  line  of  entrance  of  the  pos- 
terior nerve  roots.  These  arteries  give  off  branches  which  enter  the  cord 
to  be  distributed  more  in  the  gray  than  in  the  white  matter. 

The  spinal  arteries  originate  from  the  vertebral  arteries  which  pass 
through  the  inter-vertebral  foramina  together  with  the  spinal  roots. 

The  veins  are  situated  along  the  anterior  and  posterior  fissures  of 
the  cord  and  laterally  one  on  each  side  along  the  roots.  They  then  pass 
through  the  foramina  and  open  into  the  vertebral  veins. 

Lymph  spaces  are  found  around  the  nerve  cells  and  blood  vessels. 

MEDULLA  OBLONGATA 

Pons  and  Fourth  Ventricle 
(Rhombencephalon) 

The  medulla  or  bulb  is  the  upward  continuation  of  the  spinal  cord. 
It  occupies  the  basilar  groove  of  the  occipital  bone  with  its  lower  ex- 
tremity in  the  foramen  magnum.  Its  upper  extremity  marks  the  be- 
ginning of  the  pons. 

Its  length  is  25  mm.  (one  inch),  thickness  14  mm.  (one-half  inch) 
and  width  at  the  lower  end  one-half  inch,  at  the  upper  end  three-fourths 
of  an  inch.  Its  direction  is  almost  vertical  and  only  slightly  inclined 
forward. 

Exterior  of  the  Medulla. — It  has  an  anterior,  a  posterior  and  two  lateral 
surfaces,  also  an  upper  and  lower  extremities.  The  anterior  median 
fissure  of  the  spinal  cord  is  continued  on  the  anterior  surface  of  the  medulla, 
but  obliterated  in  its  lower  portion  on  account  of  decussation  of  the  pyra- 
mids. In  the  upper  portion  of  the  anterior  surface  are  seen  the  Pyra- 
mids. They  are  two  large  bodies'  thicker  at  the  upper  ends  than  at  the 
lower,  bounded  laterally  by  a  sulcus  from  which  emerge  the  twelfth  nerves 
(hypoglossi) .     The  sulcus  corresponds  to  a  similar  antero-lateral  sulcus 


12 


THE  CENTRAL  NERVOUS  SYSTEM 


of  the  spinal  cord  from  which  emerge  the  anterior  roots.  The  sulcus 
separates  the  pyramids  from  oval  bodies  called  Olives.  Laterally 
to  each  olive  lies  the  Restiform  body  and  in  the  sulcus  between  them 
emerge  the  roots  of  the  ninth  (glosso-pharyngeal) ,  tenth  (vagus)  and 
eleventh  (spinal  accessory)  nerves.     At  the  upper  border  of  the  pyramids 


INSULA 


ANTERIOR 

PERFORA TED  - 

SUBSTANCE 


CORPORA  MAMMIL- 
LARIA 


OLFACTORY  TRACT 
HYPOPHYSIS 

L 

fi —N  OPTICUS  (II) 

, OPTIC  TRACT 


CEREBRAL  _, 
PEDUNCLE 


GANGLION  SEMI- 
LUNARS (gasseri) 


OBLIQUE 

FASCICULUS 


TUBER  CINEREUM 

N.  OCULOMO- 
.--   TORTUS  (III) 

LATERAL 

GENICULATE 

BODY 

N.  TROCHLEARIS 
(IV) 

N.    TRIGEMINUS 
(M.  P.) 

N.   TRIGEMINUS 
(V) 

N.  ABDUCENS 
(VI) 

BRACHIUM 

PONTIS 

N.  FACIALIS 
(VII) 
PARS  INTERMEDIA 

N.  ACUSTICUS  ( VIII) 


\     N.  GLOSSO-PHARYN- 
\  GEUS  (IX  ) 


N.  HYPOGLOSSUS 


Fig. 


N.   VAGUS  (X) 

s.  N.  ACCESSORIUS  XI 
>        (spinal  accessory) 


CER  VICAL  I 


CERVICAL  II 


DECUSSATION  OF  PYRAMIDS  ' 

10. — Ventral  Aspect  of  Brain-stem  Including  Mammillary  and  Optic  Portions 


or  the  Hypothalamus.     (Morris'  Anatomy.) 


and  olives,  viz.  at  the  lower  border  of  the  pons  emerge  the  roots  of  sixth 
(abducens)  and  seventh  (facial)  nerves.  On  the  Posterior  surface  of 
the  medulla  are  seen  the  following  elements.  The  lower  portion  is  the 
closed  part  of  the  medulla,  the  upper  portion,  in  which  the  two  halves 
become  separated,  forms  an  open  triangle  as  a  part  of  the  fourth  ventricle. 


THE    CENTRAL   NERVOUS    SYSTEM 


13 


There  is  a  median  sulcus,  continuation  of  the  posterior  sulcus  of  the  cord. 
The  columns  of  Goll  end  in  an  elevation  (clava)  which  contains  a  nucleus, 


STRIA  3IEDULLARIS\ 
THALAM  y 


HABENULAR  COM- 
MISSURE 


TRIGONUNA      s 
HABENULJE   V. 


EPIPHYSIS  ,. 


MEDIAL  GEN- 
ICULATE-*^ 
BODY       - 

LATERAL  GEN-        1 
ICULATE 
BODY 


BRACHIUM  QUAD R I-  „ 
GEMINUM  INFERIOR 


CEREBRAL  PEDUNCLE 

A NTERIOR  MED  ULLARY 

VELUM 
BRACHIUM  CONJUNC- 
TIVUM 


BRACHIUM 
PONTIS 


RESTIF0R3I  BOD  Y 


CALAMUS  SCRIPTORIUS 


FUNICULUS  GRACILIS  .. 


FUNICULUS  CUNEATUS 


FUNICULUS  LATERALIS 


^-  INTERNAL  CAPSULE 
CA  UDA  TE  NUCLEUS 

SS\  \      TMNIA  CHORI- 

OIDEA 

^La.  . — STRIA  TERMIN- 
ALS THALAMI 

\ 

PULVINAR 

CORPORA  QUADRIGEMINA 

-N.  TROCHLEARIS 

LINGULA  CERE- 

BELLI 

STRIM  MEDUL- 
LARES  ACUSTICI 

TRIGONUM  VAGI  (ALA 
CINEREA  ) 

—NUCLEUS  FUNICULI  CUNEATI 

OBEX 


^  NUCLEUS  FUNICULI  GRACILIS 
(CLAVA) 


POSTERIOR  MEDIAN  FISSURE 


POSTERIOR  INTERMEDIA  TE  SULCUS 


Fig.   11. — Dorsal  Aspect  of  Medulla  Oblongata  and  Mesencephalon,  showing  the 
Floor  of  the  Fourth  Ventricle  (Rhomboid  Fossa).     {Morris,  Modified  from  Spaltehoh.) 


called  nucleus  of  Goll  (nucleus  gracilis).     Laterally  and  anteriorly  there 
is  another  elevation  containing  the  nucleus  in  which  the  fibers  of  Bur- 


14 


THE  CENTRAL  NERVOUS  SYSTEM 


dach's  columns  terminate;  it  is  called  nucleus  of  Burdach  (nucleus  cu- 
neatus). 

Laterally  are  located  the  Restiform  bodies,  which  contain  ascending 
and  descending  fibers  connecting  the  spinal  cord  with  the  cerebellum; 
they  are  the  inferior  cerebellar  peduncles  (Figs.  10,  n). 

Pons  Varolii. — It  is  a  large  mass  of  white  substance  situated  between 
the  medulla  below  and  cerebral  peduncles  (crura)  above. 


TELA  CHORIOIDEA  OF  FOURTH 

VENTRICLE 
SOLITARY  TRACT 


NUCLEUS  N.   VESTIBULI 
(VII) 

RESTIFORM  BODY 
SPINAL  TRACT  OF  TRI- 
GEMINUS 
NUCLEUS  N.  COCHLEARIS 

(VIII) 
N.   VAGUS 


N.  HYPOGLOSSUS 
PYRAMID 


—  SPINAL  TRACT  OF  TRIGEMINUS 
■  -  DECUSSATION  OF  PYRAMIDS 


Fig.  12. 


LATERAL   CEREBROSPINAL 

FASCICULUS   (crossed  pyramidal 
tract) 

.„    VENTRAL   CEREBROSPINAL 
FASCICULUS  (direct  pyramidal 
tract) 


-Diagram  showing  the  Decussation  of  the  Pyramids.     (Morris'  Anatomy.) 
The  uppermost  level  represented  is  near  the  inferior  border  of  the  pons. 


Its  anterior  surface,  containing  the  basilar  artery,  is  situated  on  the 
basilar  process  of  the  occipital  bone.  It  is  continuous  laterally  with  the 
middle  cerebellar  peduncles.  The  roots  of  the  fifth  nerve  (trigominus) 
emerge  on  both  sides  of  this  surface.  The  posterior  surface  is  continuous 
with  the  posterior  surface  of  the  medulla  and  forms  with  the  latter  the 


THE    CENTRAL   NERVOUS    SYSTEM  1 5 

floor  of  the  fourth  ventricle,  covered  by  the  cerebellum.  The  upper 
border  separates  the  pons  from  the  cerebral  peduncles.  It  corresponds 
to  sella  turcica  of  the  sphenoid  bone.  From  it  emerge  the  third  (oculo- 
motor) nerve  and  the  fourth  (pathetic)  nerve.  The  pons  consist  of  super- 
ficial and  deep  transverse  fibers  between  which  pass  the  fibers  of  the 
cerebral  peduncles  from  above  to  constitute  the  pyramids  below. 

Fourth  Ventricle. — It  is  a  rhomboidal  cavity  situated  between  the 
medulla,  pons  and  cerebellum.  It  is  continuous  below  with  the  central 
canal  of  the  spinal  cord  and  above  with  the  aqueduct  of  Sylvius. 

The  Floor. — It  is  lined  with  the  epithelium  which  is  continuous  with 
the  ependyma  of  the  central  canal.  Its  superior  half  belongs  to  the  pons, 
the  inferior  half  to  the  medulla.  It  contains  eminences  and  depressions. 
On  each  side  of  the  median  sulcus  lies  a  longitudinal  band  (funiculus 
teres)  which  commences  at  the  lower  end  (calamus  scriptorius)  as  a 
grayish  mass  (ala  cinerea) . 

Striae  Acusticae,  bundles  of  fibers  arising  in  the  nuclei  of  termination 
of  the  cochlear  division  of  the  eighth  nerve,  cross  the  floor  of  the  fourth 
ventricle  and  divide  it  into  two  halves :  upper  and  lower. 

Upper  Half. — It  contains  the  upper  portion  of  the  funiculus  teres 
(see  above),  on  each  side  of  which  lies  the  acustic  tubercle  (one  of  the 
origins  of  the  eighth  nerve).  Between  them  there  is  a  depression  (fovea 
anterior,  or  fovea  trigemini)  which  overlies  the  larger  portion  of  the  nu- 
cleus of  the  fifth  nerve.  Above  this  fovea  lies  a  grayish  mass  (locus 
cceruleus),  which  is  also  a  portion  of  the  nucleus  of  the  fifth  nerve. 

Lower  Half. — It  contains  below  at  the  origin  of  the  funiculus  teres 
ala  cinerea  (see  above),  which  corresponds  to  the  nuclei  of  the  ninth  and 
tenth  nerves.  Mesial  and  above  the  ala  cinerea  is  an  eminence  (trigo- 
num  hypoglossi)  which  corresponds  to  the  origin  of  the  twelfth  nerve. 
Externally  to  the  latter  there  is  another  eminence  (trigonum  acusticum) 
corresponding  to  the  eighth  nerve. 

Roof  of  the  Fourth  Ventricle. — The  anterior  portion  is  formed  by  the 
cerebellum  and  superior  cerebellar  peduncles.  It  is  covered  by  a  lamina 
of  white  matter,  the  anterior  medullary  velum,  the  inferior  portion  of 
which  is  continuous  with  the  white  substance  of  the  cerebellum. 

The  middle  portion  is  covered  by  the  posterior  medullary  velum, 
which  is  also  continuous  with  the  white  substance  of  the  cerebellum. 

The  inferior  portion  is  covered  by  an  epithelial  membrane  (tela  chorio- 
idea),  a  double  fold  of  the  pia  containing  vascular  processes,  viz.  choroid 
plexuses.  The  thickened  lateral  portions  of  the  tela  are  called  ligulae 
and  the  thickened  portion  of  it  at  the  calamus  is  called  obex. 

The  Margins  of  the  fourth  ventricle. 


i6 


THE  CENTRAL  NERVOUS  SYSTEM 


The  superior  borders  are  formed  by  the  superior  and  middle  cerebellar 
peduncles,  the  inferior  by  the  restiform  bodies  and  the  terminations  of 
the  posterior  columns  of  the  cord. 

Interior  of  the  Medulla  and  Pons.  — Sections  beginning  from  the 
lower  level  of  the  medulla  upward  show  the  gradual  formation  of  the 


Fig.  13. — Section  of  Medulla  Oblongata  Near  the  Pons.     (Santee.) 

a.  Hypoglossal  nucleus,  b.  Vestibular  nucleus,  c.  Tractus  solitarius.  d.  Restiform  body. 
e.  Substantia  reticularis.  /.  Hilus  of  olivary  nucleus  containing  cerebello-olivary  fibers,  g. 
Anterior  lateral  sulcus,  h.  Pyramid,  i.  Anterior  median  fissure,  j.  Anterior  longitudinal 
bundle,  k.  Medial  longitudinal  bundle  I.  Nuc.  tractus  spinalis  n.  trigemini.  m.  Tractus 
spinalis  n.  trigemini.  n.  Lateral  cochlear  nucleus.  0.  Ventral  cochlear  nucleus,  p.  Ascend- 
ing anterior  cerebello-spinal,  spino-thalamic  and  rubro-spinal  tracts,  q.  Posterior  lateral 
sulcus,  r.  Medial  fillet,  interolivary  stratum,  s.  Anterior  external  arcuate  fibers,  t.  Arcuate 
nucleus. 


Pyramids.  They  are  formed,  properly  speaking,  of  the  pyramidal  fibers 
descending  from  the  cerebral  peduncles  through  the  deep  and  superficial 
fibers  of  the  pons.  At  the  lower  level  of  the  medulla  they  begin  to 
decussate  to  go  down  in  the  cord  (see  Spinal  Cord,  Fig.  12).  Immediately 
behind  the  pyramids  lies  the  median  fillet  or  lemniscus.  This  bundle 
of  fibers  originates  in  the  nuclei  of  Goll  and  Burdach  (see  above).  As 
we  have  seen   above,    the   sensory   neurones   of    Goll's   and   Burdach's 


THE  CENTRAL  NERVOUS  SYSTEM 


17 


columns  of  the  spinal  cord  terminate  in  the  lowest  parts  of  the  medulla 
in  two  nuclei  (gracilis  or  Goll  and  cuneatus  or  Burdach).  The  function, 
however,  of  these  neurones  is  continued:  new  fibers  originate  in  the  cells 
of  these  nuclei  and  ascend.  They  begin  to  decussate  in  the  median  line 
(raphe)  with  their  fellows  of  the  opposite  side,  immediately  above  the 
decussation  of  the  pyramids  and  form  a  large  sensory  bundle,  called 
median  fillet  (lemniscus) .  At  the  level  of  the  pons  the  median  fillet 
spreads  laterally  on  both  sides;  the  lateral  portions  are  called  lateral 


GENU  OF  FACIAL  NERVE 


NUCLEUS  OF  ABDUCENS    ^ITilBUL^NERvI 


RETICULAR 
FORMATION 


DESCENDING 

ROOT  OF 

FACIAL  NERVE 


NUCLEUS  OF 
FACIAL  NERVE 


SPINAL  TRACT 
OF  TRIGEM- 
INUS 

ROOT  OF  VES- 
TIBULAR 
NERVE 
SUPERIOR 
OLIVE 


ROOT  OF  ABDUCENS 


CONTINUATION  OF 
PYRAMIDS 


Fig.  14. — Diagram  of  Transverse  Section  of  Inferior  Part  of  Pons.     {Morris,  after 

Schwalbe.) 
The  restiform  body,  not  included,  occupies  the  curved  space  lateral  to  the  nucleus  of  vestibular 

nerve. 

lemnisci.     Posteriorly  lies  a  third  bundle,  called  "posterior  longitud- 
inal fasciculus,"  the  function  of  which  is  to  associate  the  nuclei  of  the 

cranial  nerves  (Figs.  13,  14). 

In  addition  to  these  three  fasciculi  there  are  a  number  of  fibers  running 
various  courses.  Such  are  the  internal  arcuate  fibers,  some  of  which 
connect  one  restiform  body  (inferior  cerebellar  peduncle)  with  the  olivary 
body  and  the  restiform  body  of  the  opposite  side;  others  are  destined  for 
both  cerebellum  and  cerebrum. 

The  Olives  are  isolated  masses  of  gray  substance  containing  a  dense 
mass  of  fibers.  Between  them  and  the  pyramids  pass  the  twelfth  cranial 
nerves.  There  are  also  accessory  olives  situated  about  the  main  olivary 
bodies. 

The  gray  matter  of  the  medulla  and  fourth  ventricle  contains 
chiefly  nuclei  of  the  last  ten  cranial  nerves.     The  nuclei  of  the  third 


THE  CENTRAL  NERVOUS  SYSTEM 


■.;•;;  I  nucleus  of  olfa  ctor  y  ner  ve 


NUCLEUS  OF  OCULOMOTOR 
NERVE 
NUCLEUS  OF  TROCHLEAR, 
NERVE 

NUCLEUS  OF  MESENCEPHALIC 
ROOT  OF  TRIGEMINUS 


PULVINAR  OF 
\        THALAMUS 
x  LATERAL  GENIC- 
ULATE BODY 
NUCLEUS  OF  SUPE- 
RIOR COLLICULUS 


CHIEF  MOTOR  NUCLEUS  OF-- 
TRIGEMINUS 


NUCLEUS  OF  FACIAL' 

NUCLEUS  OF  ABDUCENS  *■ "  ' 

NUCLEUS  AMBIG UUS  (VAGUS 
AND  GLOSSO-PHARYNGEUS)    " 


NUCLEUS  OF  HYPOGLOSSUS  - 


NUCLEI 

OF 
OPTIC 

NERVE 


SENSORY  NUCLEUS  OF 
TRIGEMINUS 


NUCLEUS  OF  VESTIBU- 
LAR NERVE 

VENTRAL  NUCLEUS 
OF  COCHLEAR  NER  VE 


-  DORSAL  NUCLEUS  OF 
COCHLEAR  NERVE 


~~  NUCLEUS  ALJE  CINEREjE  {VAGUS 
AND  GLOSSO-PHARYNGEUS) 

-  SOLITARY  TRACT  (VAGUS  AND 
GLOSSO-PHAR  YNGEUS ) 

NUCLEUS  OF  SPINAL  TRACT  OF 
TRIGEMINUS 


NUCLEUS  OF  SPINAL  ACCESSORY 
NER  VE 


Fig.  15. — Scheme  showing  the  Relative  Size  and  Position  of  the  Nuclei  of  Origin 
(Red)  of  the  Motor  and  the  Nuclei  of  Termination  (Blue)  of  the  Sensory  Cranial 
Nerves.     (Morris'  Anatomy.) 


THE  CENTRAL  NERVOUS  SYSTEM 


19 


and  fourth  nerves  are  situated  in  the  uppermost  portion,  while  the  twelfth, 
tenth  and  eleventh  in  the  lowest  portions.  The  accompanying  illustration 
shows  sufficiently  their  anatomical  seats  without  entering  into  a  detailed 
description  (Fig.  15). 

Sections  of  the  medulla  also  show  the  seat  of  the  three  pairs  of 
cerebellar  peduncles  and  their  relation  to  the  restiform  bodies, 
which  are  the  continuation  of  the  postero-lateral  tracts  of  the  cord  and 
to  the  pons,  which  can  be  considered  as  a  continuation  of  the  middle 
cerebellar  peduncles. 


LA  TERAL  NUCLEI'S 
OF  VESTIBULAR 
NERVE 


L.  -  -  RESTIFORM  BOD  Y 


MEDIAL  BE- 

-WI—  -  SCENDING 
CEREBRO- 
PONTILE  PATH 


•.  MEDIAL 

LEMNISCUS 

CEREBRO-   {CHIEF- 
~LY  FRONTAL) 
PONTILE  RATH 


LONGITUDINAL  (PYRAMIDAL)  FASCICULI 

Fig.  16. — Diagram  showing  Connections  of  the  Fibers  of  the  Pons.     (Morris'  Anatomy.) 
The  plane  of  the  section  is  obliquely  transverse  or  parallel  with  the  direction  of  the 

brachia  pontis. 


Sections  of  the  Pons  show  the  passage  between  its  deep  and  super- 
ficial fibers  of  the  longitudinal  pyramidal  fasciculi,  which  are  the  con- 
tinuation of  the  cerebral  peduncles  (crura)  and  which  at  the  lower  border 
of  the  pons  emerge  as  pyramids  proper.  These  fibers  are  situated  in  the 
ventral  portion  of  the  pons.  In  its  dorsal  portion  (tegmentum)  the  struc- 
tures are  continuous  with  those  of  the  medulla  below  (Fig.  16). 

The  pons  also  contains  separate  aggregations  of  gray  matter,  called 
Nuclei  pontis.     They  are  dispersed  between  the  pontine  and  pyramidal 


20  THE  CENTRAL  NERVOUS  SYSTEM 

fibers.     They  receive  a  large  portion  of  these  fibers.     They  therefore 
form  the  following  connections. 

(a)  Fibers  of  cerebellar  hemispheres  end  in  the  nuclei  pontis  of  the 
opposite  side. 

(b)  Fibers  of  cerebral  hemispheres  end  in  the  nuclei  pontis,  from 
which  new  fibers  emerge  and  go  to  the  opposite  cerebellar  hemispheres. 

AREA  OF  CRURA  AND  CORPORA  QUADRIGEMINA 
(MESENCEPHALON)  (MIDDLE  BRAIN) 

It  connects  the  medulla  and  the  pons  below  with  the  forebrain  above. 

Exterior. — It  presents  a  ventral  and  dorsal  surface. 

Ventral. — It  is  formed  by  the  cerebral  peduncles  which  at  the  upper 
border  of  the  pons  present  two  thick  bundles.  Ascending  the  latter 
diverge  and  produce  the  interpeduncular  space,  the  floor  of  which  is  the 
posterior  perforated  space.  The  latter  serves  for  the  passage  of  blood 
vessels. 

The  Crura  spread  in  their  upward  direction  and  penetrate  the  brain 
under  the  optic  tracts.  Between  their  inner  surfaces  emerge  the  third 
(oculomotor)  nerves.  Their  external  surface,  which  is  covered  by  the 
Temporal  lobes,  is  surrounded  by  the  fourth  (pathetic)  nerves. 

Dorsal  Surface. — The  roof  of  the  middle  brain  is  constituted  by  a 
lamina  surmounted  by  quadrigeminal  bodies.  The  latter  present  an 
anterior  pair  and  a  posterior  pair.  Externally  each  anterior  body  is  con- 
nected by  means  of  a  white  bundle  (anterior  brachium)  with  a  ganglionic 
swelling  called  external  geniculate  body ;  a  posterior  brachium  connects 
each  posterior  quadrigeminal  body  with  an  internal  geniculate  body 
(Fig.  17). 

The  anterior  quadrigeminal  bodies  with  their  brachia  and  geniculate 
bodies  belong  to  the  optic  apparatus,  while  the  posterior  bodies  with  their 
attachments  form  a  part  of  the  auditory  apparatus. 

Posteriorly  to  the  quadrigeminal  lamina  are  seen  the  following  elements : 
(1)  a  thick-white  tract,  viz.  frenulum  of  the  anterior  medullary  velum, 
situated  between  the  anterior  quadrigeminal  bodies;  (2)  from  each  side 
of  this  tract  emerge  the  fibers  of  the  fourth  (pathetic)  nerve;  (3)  the  ter- 
mination of  the  superior  cerebellar  peduncles  which  disappear  under  the 
posterior  quadrigeminal  bodies. 

Between  the  quadrigeminal  bodies  dorsally  and  the  crura  ventrally 
lies  a  funnel-shaped  cavity,  which  connects  the  fourth  ventricle  below  and 
the  third  ventricle  in  front,  viz.  aqueduct  of  Sylvius  (Fig.  18). 

Interior  of  the  Mid-brain. — A  transverse  section  shows  a  ventral 


THE  CENTRAL  NERVOUS  SYSTEM 


21 


and  dorsal  portion.  In  the  ventral  are  seen  the  longitudinal  pyramidal 
fasciculi  covered  by  a  pigmented  stratum  of  gray  matter  (locus  niger). 
The  dorsal  portion  (tegmentum)  consists  of  red  nuclei,  in  which  apparently 
terminate  the  superior  cerebellar  peduncles,  the  gray  matter  surrounding 
the  aqueduct  of  Sylvius,  the  lamina  supporting  the  quadrigeminal  bodies 
and  the  continuation  of  other  formations  of  the  medulla. 


STRATUM ZONALB  OF  THALAMUS 

\ 

PULVINAB  OF  THALAMUS  < 


STBA  TUM  ZONALE 


NUCLEUS  OF  SUPERIOR  COLLICULUS 


MEDIAL  GENICU- 
LATE BODY  S. 


OPTIC  TRACT 


LATERAL  GENIC-  —  - 
ULATE  BODY 


RED  NUCLEUS 


EPIPHYSIS  (PINEAL  BODY) 

s     CENTBAL  GBEY 
STBATUM 


iv-—    AQU^EDUCTUS 
CEREBBI 

-  _    NUCLEUS  OF 

MESENCEPHA  LIC 
(DESCENDING) 
BOOT  OF  TBI- 
GEMINUS 

v  NUCLEUS  OF  OCULO- 
MOTOR NERVE 


MEDIAL  LONGITU- 
DINAL FASCICULUS 


INTEBPED  UNCU- 
LAR  FOSSA 


CEREBRAL  PEDUNCLE 


SUBSTANTIA  NIGRA 


FILA  OF  OCULOMOTOR  NERVE 


Fig.  17. — Transverse  Section  through  Level  oe  Superior    Quadrigeminal  Bodies. 

(Morris'  Anatomy.) 


The  median  and  lateral  lemniscus  of  the  medulla  are  here  fully  devel- 
oped. The  largest  part  of  the  latter  is  connected  with  the  nucleus  of 
the  cochlear  nerve  and  terminate  in  the  posterior  longitudinal  bodies; 
they  have  therefore  an  auditory  function.  The  median  lemniscus  (see 
Medulla)  in  its  upward  passage,  after  having  received  sensory  fibers  from 
the  nuclei  of  the  cranial  nerves,  in  reaching  the  mid-brain  sends  some 
fibers  to  the  anterior  quadrigeminal  bodies  and  terminates  in  the  Thala- 


22 


THE  CENTRAL  NERVOUS  SYSTEM 


mus  opticus  and  Hypothalamic  nucleus,  or  body  of  Luys,  which  is  situated 
immediately  below  the  lateral  and  anterior  nuclei  of  the  Thalamus. 
From  the  latter  fibers  emerge  and  go  through  the  internal  capsule  to  the 
sensory  area  of  the  cortex. 

The  posterior  longitudinal  bundles  of  the  medulla  are  found  here  in 


CENTRAL  GREY  STRATUM 


STRA  TUM  ZONALE 
\ 
NUCLEUS  OF  INFERIOR 
COLLICULUS 


AQUMDUCTUS 
CEREBRI         ->.. 
NUCLEUS  OF 
MESENCEPHA- 
LIC {DESCEND- 
ING) ROOT  OF 

TRIGEMINUS 

NUCLEUS  OF 
TROCHLEAR--- 
NERVE 

MEDIAL  LONGI- 
TUDINAL ' 

FASCICULUS 


BRACHIUM 

CONJUNC- 
TIVUM 


DECUSSATION     * 

OF  BRACHII 
CONJUNCTIVI 


POSTERIOR -i 

RECESS  OF 
INTERPEDUN- 
CULAR FOSSA 


NUCLEUS  OF  LA  TERAL  LEMNISCUS 


LEMNISCUS  {A  CO  US  TIC) 


TERAL  SULCUS  OF 
MESENCEPHALON 


MEDIAL  LEMNISCUS 


SUBSTANTIA 
NIGRA 


BASIS  OF  CEREBRAL 
PEDUNCLE 


SUPERFICIAL  FIBRES  OF  PONS 


Fig.  18—  Transverse  Section  through  the  Inferior  Quadrigeminate  Bodies.     (Morris' 

Anatomy.) 


the  most  intimate  connection  with  various  nuclei  of  nerves  supplying  the 
eye  muscles  and  other  cranial  nerves. 

The  gray  matter  is  the  continuation  of  the  same  matter  of  the  cord 
and  medulla.  Three  cranial  nerves  are  in  connection  with  the  mid-brain, 
viz.  third,  fourth  and  fifth  nerves.  The  nuclei  of  the  third  and  fourth 
form  a  continuous  column  of  nerve-cells  situated  in  the  gray  matter 
surrounding  the  aqueduct  of  Sylvius.     A  section  through  the  posterior 


THE  CENTRAL  NERVOUS  SYSTEM 


23 


quadrigeminal  bodies  shows  the  origin  of  the  fourth  nerves  and  through 
the  anterior  bodies  the  origin  of  the  third  nerves  (Fig.  19). 

The  nucleus  of  the  third  nerve  is  connected  with  the  optic  tract  by 
means  of  neurones  originating  in  the  anterior  quadrigeminal  bodies, 
with  the  fourth,  sixth  and  seventh  nerves  through  the  posterior  longi- 
tudinal bundle  (see  Medulla)  and  with  the  eighth  nerve  through  the  same 
bundle  and  lemniscus. 


usiurlz,/tos(e 


NUCLEUS  OF  POSTE- 
RIOR COMMISSURE 
AND  MED.  LONG  IT. 
FASC. 

MEDIAL  LONGITUDI- 
NAL FASCICULUS 

MM.   CILIA RIS   (a) 

AND    SPHINCTER "' 

IRIDIS  (b) 

LEVATOR     v- 

PALPEBRM 

\    3 
OBLIQUUS  INFERIOR — ~.v- ^ 

RECTUS  SUPERIOR  "-'•'•'-*£<_  :4 

RECTUS  MEDIA  LIS  —  »-.3 

RECTUS  INFERIOR  - 

(I 

(JTuA. 

'4rbckl. 


_-—  SUPERIOR  GROUP 
(Nucleus  of  Edinger 
and  Westphal) 


-INFERIOR  GROUP 


Fig.  19. — Diagram  of  Longitudinal  Section  of  Nucleus  of  Oculomotor  Nerve.     {Morris' 

Anatomy,  after  Edinger.) 


As  to  the  nucleus  of  the  fifth  (trigeminus)  nerve,  its  motor  portion 
with  the  descending  motor  fibers  is  found  in  the  mid-brain.  The  Red 
Nuclei  are  two  round  pigmented  masses  of  gray  matter,  situated  in  the 
tegmentum  under  the  anterior  quadrigeminal  bodies.  They  receive 
fibers  from  the  cerebral  cortex  and  corpus  striatum,  also  send  out  fibers 
to  the  thalamus  and  spinal  cord  (Fig.  20). 


24 


THE  CENTRAL  NERVOUS  SYSTEM 


AREA  OF  OPTIC  THALAMI,  THIRD  VENTRICLE. 
DIENCEPHALON.    INTERBRAIN 

The  Optic  Thalami  are  two  voluminous  ovoid  masses  of  gray  sub- 
stance situated  in  front  of  and  laterally  to  the  quadrigeminal  bodies  (see 
preceding  chapter)  and  on  both  sides  of  the  third  ventricle.  Close  to 
each  other  in  front  they  diverge  posteriorly. 


THALAMUS 

\-  -  INTERNAL  CAPSULE 


BUNDLE  FROM 
■     RED  NUCLEUS 
TO  THALAMUS 


'  -  BUNDLE  FROM  RED  NUCLEUS 

TO  INTERNAL  CAPSULE 
-   RED  NUCLEUS  . 
DECUSSATION  OF  BRACHIA 

CONJUNCTIVA 
.  -  BRACHIUM  CONJUNCTIVUM 
(superior  peduncle) 


INFERIOR  PEDUN- 
CLE  (resliform  body) 

BUNDLE  TO  CERE- 
--BELLAR  CORTEX 

' DENTATE 

NUCLEUS 


MEDULLA  OBLONGATA 


Fig.  20. — Transparency  Drawing  showing  the  Origin,  Course  and  Connections  of 
the  Superior  Cerebellar  Peduncles  (Brachia  Conjunctiva)  in  the  Formation  of 
"Stirling's  Scissors."     {Morris'  Anatomy.) 

The  upper  surface  (stratum  zonale)  is  white.  On  its  anterior  portion 
it  presents  an  elevation,  called  the  anterior  tubercle  or  nucleus.  The 
posterior  elevated  portion  is  called  pulvinar.  There  is  also  a  medial 
nucleus.  On  the  postero-internal  portion  of  the  same  surface  there  is 
the  trigonum  habenulse.  The  external  margin  is  separated  from  the 
adjacent  caudate  nucleus  by  a  linear  white  substance  (taenia  semicir- 
cularis). 


THE  CENTRAL  NERVOUS  SYSTEM 


25 


The  lower  surface  is  adherent  to  the  cerebral  peduncles. 

The  outer  surface  is  in  connection  with  the  internal  capsule  and 
caudate  nucleus. 

The  inner  surface  forms  the  walls  of  the  third  ventricle ;  in  its  anterior 
half  it  is  united  with  that  of  the  opposite  side  by  a  gray  commissure. 

The  Geniculate  bodies  lie  close  to  the  Thalami.     The  external  gen- 


ANTERIOR  CORNU 

OF  LATERAL 

VENTRICLE 


FIFTH  VENTRICLE 

SEPTUM 
PELLUCIDUM 

COLUMNS  OF 
FORNIX 

STRIA  TER3IINALIS 

ANTERIOR 

C03I3IISSURE 

THIRD    VENTRICLE 

MASS  A  INTER- 
MEDIA (Middle 
commissure) 
CHORIOIDAL 
SULCUS 

SUPERIOR 
COLLICULUS 
MEDIAL  GENIC- 
ULATE BODY 

LATERAL  SULCUS 

OF 
MESENCEPHA  L  ON 
PONS 

STRIA  ACUSTICA 

MEDIAN  SULCUS 

TRIG0NU3I  HYP0OL0SSI 
RESTIFORM  BODY 

CLAVA 
POSTERIOR  FISSURE 

POSTERO-INTERMEDIA  TE 
SULCUS 

POSTERO-LA  TERAL  SULCUS 


CORPUS  CALLOSUM 


NUCLEUS 
CA  UDA  TUS 

FORAMEN  INTER- 
VENTRICULARS 
(Monroi) 

ANTERIOR   TUBER 
CLE  OF  THALA- 
31  US 

STRIA  MEDUL- 
LARS THALA31I 

HABENULAR  C03I- 
3IISSURE 

EPIPHYSIS 

SULCUS  CORP. 

QUAD.  31  EDI  A  LIS 

INFERIOR 
COLLICULUS 

FRENULUM  VELI 

LINGULA 
CEREBELLI 

FACIAL  E3IINENCE 


AREA  ACUSTICA 

TRIG0NU3I  VAGI 

TUBER  CUL  U3I  CUNEA  TU31 
FUNICULUS  GRACILIS 
FUNICULUS  CUNEA  TUS 
LATERAL  FUNICULUS 


Fig.    21. — Dorsal  Surface  of  Diencephalon  with  Adjacent  Structures  (Morris,  after 

Obersteiner .) 


iculate  body  is  closely  attached  to  the  posterior  extremity  or  base  of  the 
Thalamus  (pulvinar)  and  through  the  anterior  brachium  is  connected 
with  the  anterior  quadrigeminal  body  (Fig.  21). 

Third  Ventricle. — It  is  a  single  cavity  situated  between  the  optic 
thalami,  beneath  the  fornix.     The  floor  is  represented  from  the  anterior 


2  6  THE  CENTRAL  NERVOUS  SYSTEM 

angle  backward  by  the  posterior  perforated  space,  mammillary  bodies, 
tuber  cinereum,  optic  chiasma. 

The  lateral  surfaces  are  formed  by  the  thalami. 

The  anterior  border  extends  from  the  foramen  of  Monro  down  to  the 
optic  chiasma  and  contains  the  two  anterior  pillars  of  the  fornix  between 
which  lies  the  anterior  white  commissure. 

The  roof  is  formed  of  the  tela  chorioidea  covered  by  the  fornix,  upon 
which  lies  the  corpus  callosum.  The  third  ventricle  communicates 
posteriorly  with  the  aqueduct  of  Sylvius  and  anteriorly  through  two 
openings  (foramina  of  Monro)  with  the  lateral  ventricles. 

In  connection  with  the  thalamic  area  must  be  mentioned  the  Epithala- 
mus  or  Epiphysis  (pineal  body)  and  the  Hypophysis  (pituitary  gland). 

Epithalamus  or  Epiphysis  (pineal  body). — It  develops  on  the  roof  of 
the  third  ventricle.  It  presents  an  ovoid  body  of  10  mm.  long,  situated 
posteriorly  at  the  entrance  of  the  third  ventricle  in  the  groove  between 
the  anterior  quadrigeminal  bodies.  It  is  fixed  by  its  adherence  to  the 
pia-mater  and  by  its  continuity  with  the  walls  of  the  third  ventricle. 
From  its  base  appear  two  bands  (strias  pinealis)  which  extend  anteriorly 
upon  the  upper  border  of  the  third  ventricle,  one  on  each  side.  Just 
below  and  lateral  to  the  epiphysis  there  is  a  small  group  of  nerve  cells, 
called  the  habenular  nucleus. 

Hypophysis  or  Pituitary  Body. — It  lies  in  the  sella  turcica  of  the 
sphenoid  bone.  It  consists  of  two  portions :  a  large  anterior  or  glandular 
lobe  and  a  posterior  or  cerebral  lobe.  The  infundibulum  is  continuous 
only  with  the  posterior  lobe. 

The  glandular  portion  originates  from  a  diverticulum  of  the  buccal 
cavity.  The  cerebral  portion  comes  from  the  floor  of  the  third  ventricle. 
The  first  is  ascending  and  the  second  is  descending  in  development. 

The  other  portions  belonging  to  the  interbrain  are:  optic  chiasma, 
tuber  cinereum  and  infundibulum.  The  latter  is  the  apex  of  the  tuber 
cinereum.     They  are  all  parts  of  the  floor  of  the  third  ventricle. 

CEREBRAL  HEMISPHERES.     TELENCEPHALON 

They  are  two  symmetrical  masses  of  nervous  tissue,  ovoid  in  shape. 
They  occupy  the  cranial  cavity.  Their  surface  is  gray  (cortex).  The 
average  length  is  16  cm.,  width  13  cm.,  height  12  cm.  They  present  a 
Convexity  and  Base. 

A  longitudinal  fissure  separates  the  two  hemispheres.  From  the 
cerebellum,  over  which  lie  the  occipital  lobes,  and  from  the  mesencephalon 
(see  above)  they  are  separated  by  a  transverse  fissure.  Each  hemisphere 
presents  an  external,  mesial  and  inferior  surface. 


THE  CENTRAL  NERVOUS  SYSTEM 


27 


A  number  of  fissures  or  sulci  divide  each  hemisphere  into  lobes,  lobules, 
convolutions.  The  adjoining  illustrations  are  sufficient  to  give  a  correct 
idea  of  the  division  and  subdivision  of  the  cerebral  cortex  by  the  fissures, 
also  of  the  Base  of  the  Brain  with  the  cranial  nerves  (Figs.  22,  23,  24,  25, 
26,  27). 

As  to  the  function  of  the  various  areas,  see  the  chapter  on  Localizations. 


Fig.  22. — Gyri  of  Convex  Surface  of  Left  Cerebral  Hemisphere.     (Santee.) 

Fissura  lateralis  cerebri:  a.  Stem.  b.  Horizontal  anterior  ramus,  c.  Ascending  anterior 
ramus,  d.  Posterior  ramus,  e,  e.  Sulcus  centralis  (Rolandi).  /.  Genu  superius.  g.  Genu 
inferius.  h.  Sul.  occipito-parietalis.  i,i,i.  Sul.  frontalis  superior.  j,j.  Sul.  frontalis  inferior. 
k,  k.  Sul.  frontalis  medius.  I,  I.  Sul.  paramedialis.  m,  m.  Sul.  praecentralis  inferior,  n,  n 
Sul.  praecentralis  superior.  0.  Sul.  post-centralis  inferior,  p.  Sul.  post-centralis  superior,  q. 
Ramus  horizontals  and  r,  ramus  occipitalis  of  interparietal  sulcus.  5.  Sul.  transversus.  /. 
Sulci  superior  and  lateralis,  u.  Incisura  prasoccipitalis.  v.  Sul.  temporalis  superior,  w.  SuL 
temporalis  medius. 

Interior  of  the  Brain. — A  horizontal  section  through  both  hemispheres 
reveals  the  presence  of  gray  and  white  matter,  also  of  lateral  ventricles. 

Gray  Substance. — Besides  the  cortical  gray  matter  there  are  also 
isolated  masses  called  basal  ganglia.  The  latter  are  three  in  number, 
viz.  Thalamus,  Caudate  and  Lenticular  nuclei.  The  two  last  ones  are  also 
known  under  the  name  of  Corpora  striata.  The  thalamus  was  discussed 
in  the  preceding  chapter  (Fig.  28). 

Caudate  Nucleus  belongs  to  the  wall  of  the  lateral  ventricle.  It 
forms  the  floor  in  the  upper  portion  and  the  roof  in  the  lower  portion  of 


28  THE    CENTRAL    NERVOUS    SYSTEM 

CENTRAL  SULCUS  (ROLANDI) 


PARIETAL   LOBE 


FRONTAL  LOBE 


OCCIPITAL  LOBE 


CEREBELLUM 


CENTRAL  LOBE  {INSULA) 

TEMPORAL  LOBE 


PONS 


CENTRAL  SULCUS  OP  INSULA 

Fig.  23. — Diagram  of  the  Convex  Surface  of  thf  Left  Cerebral  Hemisphere  showing 

the  FrvE  Principal  Lobes  of  the  Pallium.     (Morris'  Anatomy.) 
The  opercular  regions  of  the  frontal,  pacietal,  and  temporal  lobes  are  removed  to  show  the 

central  lobe  or  island  of  Reil. 


Fig.  24. — Latero-superior  Vtew  of  Brain,  showing  Fissures  and  Lobes.     (Santee.) 
a.  Longitudinal  fissure,   b.  Frontal  pole.    c.  Temporal  pole.    d.  Impressio  petrosa.    e.  Occipito- 
parietal sulcus,     f.  Occipital  pole. 


THE  CENTRAL  NERVOUS  SYSTEM 


29 


the  lateral  ventricle.  The  anterior  portion  (head)  is  closely  connected 
with  the  internal  capsule,  the  body  is  applied  to  the  optic  thalamus,  the 
posterior  portion  passes  around  the  posterior  border  of  the  capsules  to 


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go  down  into  the  inferior  cornu  of  the  lateral  ventricle  and  then  forward 
to  the  apex  of  the  temporal  lobe. 

The  Lenticular  Nucleus  is  an  extra-ventricular  body.  It  is  situated 
between  the  thalamus  and  insula,  externally  and  beneath  the  caudate  nu- 
cleus.    It  is  separated  externally  from  the  insula  by  a  layer  of  white 


3° 


THE  CENTRAL  NERVOUS  SYSTEM 


substance,  called  External  Capsule  and  the  intervening  claustrum.  The 
internal  surface  is  in  relation  with  the  thalamus,  caudate  nucleus  and 
internal  capsule.     The  lenticular  nucleus  is  divided  by  two  vertically 


curving  lamina  into  three  portions,  viz.  globus  pallidus  for  the  two  inner 
zones  and  putamen  for  the  outer  zone. 


THE  CENTRAL  NERVOUS  SYSTEM 


31 


White  substance  of  the  hemispheres  is  represented  mainly  by  the 
following  portions:  Corpus  callosum,  Internal  capsule,  Corona  radiata, 
Association  fibers,  Fornix. 

Corpus  Callosum. — It  is  a  wide  white  commissure  connecting  hori- 
zontally both  hemispheres.  It  is  curved  antero-posteriorly  and  covers 
the  lateral  ventricles.  The  anterior  extremity  or  knee  in  curving  anteriorly 
embraces  the  striate  bodies  and  closes  the  lateral  ventricles.  Lower 
down  it  becomes  thin,  and  under  the  name  of  rostrum  reaches  the  con- 
volution of  the  corpus  callosum  (gyrus  fornicatus).  The  posterior  ex- 
tremity (splenium)  lies  on  the  quadrigeminal  bodies.     The  upper  surface 


GYRUS  CINGULI  , 

MEDIAL  AND  LATERAL 
LONGITUDINAL  STRIJE 
OF  CORPUS  CALLOSUM 

SEPTUM  PELLUCIDUM 


SUBCALLOSAL  GYRUS 
OLFACTORY  BULB 


MEDIAL  OLFACTORY  STRIA 


LATERAL  OLFACTORY  STRIA 


THA  LA  MO-MA  MMILLA  R  Y 
FA  SCICUL  US     (  VICQ 
D'AZYRl) 
CORPUS  MAMMILLARE 


dentate  fascia  or  gyrus 
Fig.  27. — Diagram  showing  Position  of  Structures  Comprising  the  Limbic  Lobe  as 
Seen  from  the  Mesial  Aspect  of  the  Cerebral  Hemisphere.     {Morris'  Anatomy.) 


is  in  relation  with  the  falx  cerebri,  the  inferior  border  of  which  contains 
the  inferior  longitudinal  sinus.  Laterally  it  is  covered  by  the  gyrus 
fornicatus.  The  inferior  surface  lies  on  the  septum  lucidum  in  front  and 
on  the  fornix  posteriorly. 

Internal  Capsule. — It  is  a  mass  of  white  substance  situated  between 
the  lenticular  nucleus  on  its  outer  side  and  the  caudate  nucleus  and  thala- 
mus on  its  inner  side.  Its  direction  is  upward  and  outward.  It  has  an 
anterior  limb  (between  the  caudate  and  lenticular  nuclei)  directed  forward 
and  outward,  a  posterior  limb  (between  the  lenticular  nucleus  and  thala- 
mus) directed  backward  and  outward,  and  a  knee,  the  angle  where  the 
two  limbs  meet. 

Corona  Radiata. — The  fibers  of  the  internal  capsule,  after  they  leave 
the  surrounding  basal  ganglia,  as  well  as  the  fibers  of  the  corpus  callosum, 
spread  in  all  directions  to  the  cortex  (centrum  semiovale) .  The  radiations 
of  the  internal  capsule  are  called  corona  radiata.     Among  the  latter  there 


32 


THE  CENTRAL  NERVOUS  SYSTEM 


is  a  special  posterior  band  distributed  in  the  lateral  wall  of  the  posterior 
cornu  of  the  lateral  ventricle,  called  optic  radiations.  They  originate  in 
the  cells  of  the  pulvinar,  lateral  geniculate  body  and  anterior  quad- 
rigeminal  body. 

The  entire  white  substance  of  the   brain  presents  three  systems  of 


ANTERIOR  CORNU 


GYRUS  CINGULT 


CORPUS  CALLOSUM 


CA  UDA  TE 
NUCLEUS 
INTERNAL  CAP- 
SULE (FRONTAL 
PORTION) 

EXTERNAL 

CAPSULE 

LENTICU-  f  PVTAMEN 

LAR  NU-<        GLOBUS 

CLEUS      I  PALLID  US 

CIA  USTR  UM 
INTERNAL  CAP- 
SULE [OCCIPITAL 
PORTION) 

THALAMUS 


MEDIAL   GENI- 
CULATE BODY 
CA  UDA  TE 
NUCLEUS 
HIPPOCAMPUS 

MAJOR 

HIPPOCAMPUS 

MINOR 


SEPTUM 
PELLUCID  UM 

CORPUS 

STRIATUM 
COLUMN  OF 

FORNIX 

STRIA  TERMI- 
NALS OF 
THALAMUS 

THALAMUS 


CORPORA  QUAD- 
R1GEMINA 


BRACHIUM    \ 
CONJUSC- 
TIYUM         t 

REST/FORM ' 
BODY  ( 

BRACHWit    ' 
PONTJS       j 


CERE- 
BELLAR 
PEDUN- 
CLES 


TRIGONUM  VAGI 
(ALA  CINEREA) 


CLA  VA 
FUNICULUS  CUNEATUS 

FUNICULUS  GRACILIS 


Fig.  28. — Horizontal  Dissection  showing  the  Gray  and  White  Substance  of  the 
Telencephalon  Below  the  Corpus  Callosum  and  the  Relative  Position  of  the  Thal- 
amencephalon.     {Morris,  after  Landois  and  Stirling.) 


fibers  having  different  functions.     They  are:  projection,  association  and 
commissural  fibers. 

A.  Projection  Fibers. — They  are  ascending  and  descending. 

Ascending  fibers  are  constituted  by  the  following  tracts  (Fig.  2 9  ). 


THE  CENTRAL  NERVOUS  SYSTEM 


33 


CORONA 
RAUIATA 


INTERNAL 
■  CAPSULE 


HYPO- 

■  THALAMIC 
NUCLEUS 


MEDIAL 

LEMNISCUS 


AFFERENT  OR 
--SENSORY  CRA- 
NIAL NERVE 


NUCLEUS  OF  FUNICU- 
LUS CUNEATVS 

NUCLEUS  OF  FUNICU- 
LUS GRACILIS 


-FASCICUL  US  CUNEA  TUS 


FASCICULUS  GRACILIS 


>-  SPINAL  GANGLIA 


Fig.  29. — Scheme    of    Ascending    Cerebro-spinal    Conduction    Pathways.     {Morris' 

Anatomy.) 
3 


34 


SOMJESTHETIC 
AREA    OF 
CEREBRAL 
CORTEX 


CA  UDA  TE 
NUCLEUS 


INTERNAL 
CAPSULE 


LENTICULAR 
NUCLEUS" 


CEREBRAL 

PEDUNCLE 

TROCHLEAR 

NER  VE 


THE    CENTRAL   NERVOUS    SYSTEM 


MEDULLA  OBLONGATA 


MOTOR 
NUCLEI 
OF 

CRANIAL 
NER  VES 


DECUSSATION  OF 
PYRAMIDS 


LATERAL  CEREBRO- 
SPINAL FASCICULUS 


VENTRAL  CEREBRO- 
SPINAL FASCICULUS 


VENTRAL  ROOTS  OF 

SPINAL  NERVES 
VENTRAL   WHITE  COM- 
MISSURE 


SPINAL  CORD 


Fig.  30.— Scheme    of    Descending    Cerebro-spinal    Conduction   Pathways.     (Morris' 

Anatomy.) 


THE  CENTRAL  NERVOUS  SYSTEM 


35 


1.  Sensory,  which  are  the  continuation  of  the  median  lemniscus  (see 
Medulla).  They  terminate  in  the  thalamus  and  subthalamic  nucleus, 
from  which  new  fibers  emerge  and  go  through  the  posterior  portion  of  the 
posterior  limb  of  the  internal  capsule  to  terminate  in  the  sensory  area  of 
the  cortex.  The  median  lemniscus  is  joined  by  fibers  from  the  sensory 
nuclei  of  the  cranial  nerves. 

2.  The  cochlear  branch  of  the  eighth  nerve  also  sends  projection 
fibers  through  the  lateral  lemniscus  to  the  posterior  limb  of  the  internal 
capsule,  from  which  fibers  go  to  the  cortex  of  the  temporal  lobe. 


FIBRM  PROPRIA 


SUPERIOR  LONGITUD- 
INAL FASCICULUS 
STRIA   TERMINALIS         t 
OF  THALAMUS 


CINGULUM 


UN  CIS  A  TE 
FASCICUL  US 


INFERIOR  LONGITUDINAL  FASCICULUS 


Fig.  31  — 


Schematic  Representation  or  Certain  of  the  Association  Pathways  of  the 

Cerebral  Hemisphere.     (Morris'  Anatomy.) 


3.  The  optic  radiations  are  mentioned  above. 

4.  The  superior  cerebellar  peduncles  terminate  mostly  in  the  red  nuclei 
(see  Mesencephalon  and  Thalami),  from  which  new  fibers  ascend  and  go 
to  the  sensory  area  of  the  cortex. 

Descending  Fibers  are  the  following  (Fig.  30). 

1.  Pyramidal  fibers,  which  originate  in  the  cells  of  the  motor  area,  pass 
through  the  largest  part  of  the  posterior  limb  adjacent  to  the  knee  and 
through  the  latter,  down  to  the  crura,  pyramids,  and  after  decussating 


36 


THE  CENTRAL  NERVOUS  SYSTEM 


in  the  medulla  descend  in  the  cord  and  terminate  around  the  cells  of  the 
anterior  cornua. 

2.  Temporal  Bundle  originates  in  the  cortex  of  the  first  two  temporal 
gyri  and  passing  through  the  posterior  limb  of  the  internal  capsule  descend 
into  the  lateral  portion  of  the  crura  and  thence  to  the  nuclei  of  the  pons. 

3.  Frontal  Bundle  originates  in  the  cortex  of  the  frontal  lobe  and 


Fig.  32. — Dorsal  Surface  of  Corpus  Callosum,  Cerebral  Hemisphere  Cut  Away  to 

Expose  It.     (Santee.) 


passing  through  the  anterior  limb  of  the  internal  capsule  ends  in  the  nuclei 
of  the  pons. 

4.  Occipital  Bundle  (visual)  originates  in  the  cortex  of  the  cuneus  and 
calcarine  fissure  and  passing  through  the  most  posterior  portion  of  the  in- 
ternal capsule  ends  in  the  anterior  quadrigeminal  body. 


THE  CENTRAL  NERVOUS  SYSTEM 


37 


B.  Association  Fibers. — They  connect  different  parts  of  the  same 
hemisphere.     They  are  the  following  (Fig.  31). 

1.  Superior  Longitudinal  Bundle  which  connects  the  frontal,  temporal 
and  occipital  lobes. 

2.  Inferior  Longitudinal  Bundle   which   connects   the  occipital  and 
temporal  lobes. 


CA  UDA  TE 

NUCLEUS 


ANTERIOR 

PILLARS 

OF  FORNIX 


Veins  of  Galen 


POSTERIOR 

PILLARS 

OF  FORNIX 


Straight  si?nis 


CERE- 
BELLUM 


CORPUS 
CALLOSUM 
(in  section) 

SEPTUM 

-  PELLUCI- 
BUM 

FIFTH 
VENTRICLE 

STRIA    TER- 
MINALS 
OF  THALA- 
MUS 

THALAMUS 

Tela 
ehorioidea 
(velum  inter - 
positum) 

Chorio  id  plexus 

-  FIMBRIA 
HIPPOCAM-_ 

f^H  EMINENTIA 
COLL  A  TER- 
ALIS 

HIPPO- 
CAMPUS 
MINOR 


Fig.  2,2,. — Horizontal  Dissection  of  the  Cerebral  Hemispheres. 
The  fornix  has  been  removed  to  show  the  relation  of  the  tela  ehorioidea  of  the  third  ven- 
tricle to  the  chorioid  plexus  of  the  lateral  ventricles.     (From  a  mounted  specimen  in  the 
Anatomical  Department  of  Trinity  College,  Dublin.) 

3.  Occipito -frontal  Bundle. 

4.  Fibrse  propriae  connecting  contiguous  gyri  with  each  other. 

5.  Uncinate  Bundle  connects  the  uncus  with  the  frontal  lobe. 

6.  The  Cingulum  (see  illustration). 

C.  Commissural  Fibers. — They  connect  one  hemisphere  with  the  other. 
They  consist  of 


38  THE  CENTRAL  NERVOUS  SYSTEM 

i.  Corpus  Callosum  (see  above,  Fig.  32). 

2.  Anterior  commissure, the  largest  part  of  which  connects  one  tem- 
poral lobe  with  another. 

3.  Hippocampal  commissure  connects  the  two  gyri  of  the  same  name. 
Lateral  Ventricles. — Each  of  these  cavities  commences  within  the 

frontal  lobe,  is  directed  backward,  turns  around  the  optic  thalamus  to  go 
again  forward  and  downward  and  terminate  in  the  apex  of  the  temporal 
lobe.     It  presents  an  anterior,  posterior  and  inferior  cornu  and  a  central 


CORP.  GEN. INT. 


Fig.  34. — Diagram  of  Principal  Pathways  of  Optic  Apparatus. 

ham.) 


{Morris,  after  Cunning- 


portion  or  body.  Its  relation  to  various  portions  of  the  brain  has  been 
already  mentioned.  It  communicates  with  the  third  ventricle  by  means 
of  the  foramen  of  Monro  situated  in  the  frontal  portion  of  the  latter.  The 
tela  choroidea  of  the  third  ventricle  is  continuous  into  the  lateral  ventricles 
and  the  varicose  mass  composed  of  blood  vessels  found  in  the  lateral  ven- 
tricles is  known  under  the  name  of  choroid  plexus.     The  anterior  portions 


THE    CENTRAL    NERVOUS    SYSTEM 


39 


TEGMENTUM 


FRENULUM  VELI 


CEREBRAL  PEDUNCLE 
SUBSTANTIA  NIGRA 


INFERIOR  QUADRIGEMIN- 
ATE  BODY 


CENTRA  E 
LOBULE 


ALA  OF  CENTRAL 
LOBULE 

ANTERIOR  SEMI- 
LUNAR LOBE 


POSTERIOR 

SEMILbNAR 

LOBE 

SUPERIOR  — 

SEMILUNAR        ' 

LOBE 


HORIZONTAL 
FISSURE 


INFERIOR 
SEMILUNAR  m      '  -^ssgysjs-  CLIVUS  OF  MONTICULUS 

LOBE 

POSTERIOR  CEREBELLAR  NOTCH  FOLIUM  OF  VERMIS 

Yig.  35.— Diagram  of  the  Dorsal  Surface  of  the  Cerebellum.     {Morris''  Anatomy.) 


SUPERIOR  CEREBEL- 
LAR PEDUNCLE 
POSTERIOR  MEDUL- 
LARY VELUM 
MIDDLE  CEREBELLAR 
PEDUNCLE 


FLOCCULUS 


ANTERIOR 
FOURTH    MEDULLARY 
VENTRICLE     VELUM    L1NGULA 

NODULE 


UVULA 


B I  VENTRAL 
LOBE 

LOBULUS 

GRACILIS 

ANTERIOR     . 

LOBULUSS 

GRACILIS 

POSTERIOR 

INFERIOR. 

SEMILUNAR 

LOBE 


TONSIL 


TUBER  VERMIS 


PYRAMID 
POSTERIOR  CEREBELLAR  NOTCH 


Fig.  36. — Diagram  of  the  Ventral  and  Inferior  Surface  of  the  Cerebellum  after 

the  Removal  of  the  Medulla  Oblongata,  Pons,  and  Mesencephalon. 

(Morris'  Anatomy.) 

The  tonsil  of  the  right  side  is  omitted  in  order  to  display  the  connection  of  the  pyramid 

with  the  biventral  lobe,  the  furrowed  band  of  the  uvula,  and  more  fully  the  posterior  medullary 

velum.     The  anterior  notch  is  less  evident  than  in  the  actual  specimen. 


40  THE  CENTRAL  NERVOUS  SYSTEM 

of  the  two  lateral  ventricles  are  separated  from  each  other  by  the  Septum 
lucidum,  a  thin  vertical  membrane  attached  in  front  to  the  corpus 
callosum  and  the  anterior  pillars  of  the  fornix.  It  consists  of  two 
layers,  between  which  there  is  a  closed  cavity  called  fifth  ventricle,  which 
has  no  communication  with  other  ventricles  (Fig.  33). 

Fornix. — It  is  placed  beneath  the  corpus  callosum.  It  extends  from 
the  splenium  to  the  anterior  portion  of  the  corpus  callosum,  to  which  it  is 
attached  by  the  septum  lucidum.  It  presents  a  body  and  anterior  and 
posterior  pairs  of  bands  (columns  or  pillars).  The  anterior  pillars  run 
forward  and  downward,  and  appear  at  the  base  of  the  brain  as  mammillary 
bodies.  The  posterior  pillars  curve  backward,  downward  and  forward 
and  end  in  the  uncus.  The  fornix  is  an  association  tract  of  the  limbic 
lobe.  The  posterior  pillars  connect  the  hippocampal  gyri  by  means  of  a 
lamina  situated  between  them.  Fibers  emanating  from  the  mammillary 
bodies  (anterior  pillars)  go  to  the  thalamus  and  crura  of  the  same  and 
opposite  side. 

OPTIC  APPARATUS 

In  discussing  the  structure  of  the  brain  various  portions  of  white  and 
gray  matter  were  mentioned  in  connection  with  the  visual  apparatus. 

A  recapitulation  of  those  elements  will  be  useful  in  this  place.  The 
adjoining  illustration  gives  a  satisfactory  idea  of  the  entire  optic  apparatus 
(Fig.  34). 

CEREBELLUM 

It  lies  in  the  posterior  fossae  of  the  cranium,  under  the  occipital  lobes 
of  the  cerebrum,  behind  the  medulla  oblongata. 

Exterior. — Similarly  to  the  cerebrum,  the  cerebellum  is  divided  by 
fissures  and  sulci  into  lobes  and  lobules  (Figs.  35  and  36). 

The  adjoining  illustrations  give  a  sufficiently  clear  idea  of  the  external 
appearance  of  the  cerebellum. 

Interior. — A  section  of  the  cerebellum  reveals  in  each  hemisphere  a 
gray  cortical  mass,  central  white  matter  and  several  ganglionic  masses 
of  gray  substance  (Figs.  37  and  38). 

Gray  Matter.— It  is  presented  by  the  Dentate  nucleus  and  accessory 
nuclei. 

The  Dentate  nucleus,  situated  in  the  center  of  the  white  substance, 
presents  an  ovoid  and  folded  lamina  with  an  opening  (hilus) .  It  resembles 
the  olivary  bodies  of  the  medulla. 

Three  accessory  small  nuclei  are  situated  near  the  dentate  nucleus. 

White  Matter.— In  addition  to  the  central  white  mass,  there  are  also 


THE  CENTRAL  NERVOUS  SYSTEM 


41 


Fig.  37. — Median  Section  of  Cerebellum,  Pons  and  Medulla.  (Santee.) 
a.  Predeclivil  sulcus,  b.  Arbor  vita?,  c.  Declive  monticuli.  d.  Postdeclivil  sulcus,  e. 
Folium  vermis.  /.  Horizontal  sulcus,  g.  Tuber  vermis.  /;.  Postpyramidal  sulcus,  i. 
Pyramid,  j.  Prepyramidal  sulcus,  k.  Uvula.  I.  Culmen  monticuli.  m.  Postcentral  sulcus. 
n.  Central  lobule.  0.  Inferior  colliculus  of  corp.  quad.  p.  Cerebral  aqueduct,  q.  Precentral 
sulcus,  r.  Superior  medullary  velum,  s.  Lingula.  t.  Medial  longitudinal  bundle,  u.  Fasti- 
gium.     v.  Inferior  medullary  velum,     w.  Nodule,     x.  Postnodular  sulcus. 


Fig.  38. — Sagittal  Section  of  Cerebellum,  Cutting  Nucleus  Dentatus.  (Santee.) 
a.  Sup.  semilunar  lobule,  b.  Corpus  medullare.  c.  Post,  part  quadrangular  lobule,  d. 
Nucleus  dentatus.  e.  Ant.  part  of  quadrangular  lobule.  /.  Interior  of  dentate  nuc.  g. 
Central  sulci,  h.  Bracnium  pontis.  i.  Restiform  body.  j.  Inf.  semilunar  and  slender  lobules. 
k.  Hilus  of  nuc.  dent.     I.  Biventral  lobule. 


42 


THE  CENTRAL  NERVOUS  SYSTEM 


DECUSSATION  OF  BRACHIA 
CONJUNCTIVA 


DENTA  TE 
,    NUCLEUS 


CORONA 
RADIATA 

THALAMUS 
LATERAL  NUCLEUS 
OF  THALAMUS 

■    INTERNAL 
CAPSULE 


RED  NUCLEUS 


VERMIS 
OF  CERE 
BELL UM 

(displaced } 


~-—    AFFERENT  CRANIAL 
'■"    NERVES  (VESTIBULAR 
CHIEFLY) 


EXTERNAL  ARCUATE  FIBRES  < 


NUCLEUS  OF  FUNICULUS 

CUNEATUS 

NUCLEUS  OF  FUNICULUS 

GRACILIS 


SPINAL   GANGLIA 


Fig.  39. — Scheme  of  Principal  Ascending  Cerebellar   Conduction  Paths.      {Morris' 

Anatomy.) 


THE  CENTRAL  NERVOUS  SYSTEM 


43 


three  pairs  of  peduncles  uniting  the  cerebellum  with  the  brain,  mid-brain 
and  spinal  cord.  The  situation,  course  and  termination  of  the  cerebellar 
peduncles  have  been  sufficiently  discussed  in  the  study  of  each  portion  of 
the  central  nervous  system  (see  also  illustrations,  Figs.  39  and  40). 


Fig.  40. — Scheme  of  Cerebellar  Connections.     {From  Poirier  and  Charpy.) 

Efferent  or  centrifugal  fibers  in  red.     Afferent  or  centripetal  fibers  in  blue.     Small  circles  in 

the  center  are  pontine  nuclei. 

MENINGES  OF  THE  BRAIN 

The  brain  is  surrounded  by  three  membranes,  viz.  dura-mater,  arach- 
noid and  pia-mater.     (Fig.  41). 

Dura. — It  consists  of  two  layers:  an  outer  which  serves  as  endosteum 


44 


THE  CENTRAL  NERVOUS  SYSTEM 


of  the  cranium,  and  an  inner  layer  which  sends  processes  between  subdi- 
visions of  the  brain. 

The  outer  layer  adheres  to  the  cranium,  especially  along  the  sutures 
and  at  the  base. 

The  inner  layer  gives  off  prolongations,  three  in  number,  viz.  falx 
cerebri,  falx  cerebelli,  tentorium  cerebelli. 


FOURTH  NERVE 


Falx  cerebri 


THIRD  NERVE 

SECOND  NERVE 


Superior  sagit- 
tal sinus 

Inferior  sagit- 
tal sinus 


Vein  of  Galen 


Straight  sinus 


Superior 
petrosal  sinus 

Falx  cerebelli 

SEVENTH  AND 
EIGHTH 
NERVES 
NINTH,  TENTH,  AND 
ELEVENTH  NER  VES 

TWELFTH  NER  VE 

SECOND  CERVICAL  NERVE 

Ligamentum  denticulatum 


Vertebral 
artery 


N 

FIRST 
CERVICAL 

NER  I  'E    Inferior  petrosal  sinus 


SIXTH         FIFTH 
NERVE        NERVE 


Fig.  41. — The  Cranium  with  Encephalon  Removed  to  show  the  Falx  Cerebri,  the 
Tentorium  Cerebelli,  and  the  places  where  the  Cranial  Nerves  Pierce  the  Dura 
Mater.     (Morris,  after  Sappey.) 

Falx  cerebri  is  a  vertical  projection  in  the  longitudinal  fissure  attached 
in  front  to  the  crista  galli,  behind  to  the  upper  surface  of  the  tentorium 
cerebelli. 

Falx  cerebelli  is  a  smaller  process  of  the  dura  than  the  preceding  one, 
which  is  inserted  between  the  hemispheres  of  the  cerebellum.  It  is  at- 
tached behind  to  the  internal  occipital  crest  and  above  to  the  tentorium 
cerebelli. 

Tentorium  cerebelli  is  an  arched  tent-like  process  between  the  cere- 
brum and  cerebellum. 

The  two  layers  of  the  dura  become  separated  in  certain  places  to  form 
venous  sinuses.     They  are  fifteen  in  number — five  paired  and  five  single. 


THE    CENTRAL    NERVOUS    SYSTEM 


45 


The  paired  ones  are  two  lateral,  two  superior  petrosals  and  two  inferior 
petrosals,  two  cavernous  and  two  occipitals.  The  single  sinuses  are  the 
superior  longitudinal,  inferior  longitudinal,  the  straight,  the  circular  and 
the  transverse. 

The  accompanying  illustrations  give  a  satisfactory  idea  of  their  re- 
spective seats  (Figs.  41,  42  and  43). 

Arachnoid.— It  is  situated  between  the  dura  and  pia.  It  envelops  the 
brain  but  does  not  penetrate  the  fissures.     At  the  level  of  the  fissures  into 


1MIXDIBULUM 

Internal  carotid  artery . 

CRISTA  GALLI 

OPTIC  NERVE 


Spheno-pariela!  sinus 


Middle  cerebral 

artery 
Anterior  cerebral 
artery 

Posterior  communi- 
cating artery 
Cavernous  sinus 


OPTIC  TRACT 
THIRD  NERVE 
Cerebral  peduncle 

Aqueduct  of  Sylvius 
MESENCEPH- 
ALON 

Falx  cerebri 

Tentorium  cerebell 
Straight  sinus 


Superior  cerebellar 

artery 
Posterior  cerebral 

artery 
Superior  petrosal 

sinus 

Free  border  of  ten- 
torium bounding 
tentorial  notch 


Transverse  sinus 


Superior  sagittal  sinus 


Fig.  42. — Showing  the  Upper  Surfaces  of  the  Tentorium  Cerebelli  and  the  Ten- 
torial Notch  through  which  the  Mid-brain  and  Posterior  Cerebral  Arteries  Enter 
the  Middle  Fossa  of  the  Cranium.     (Morris'  Anatomy.) 


which  the  pia  dips  the  two  membranes  are  widely  separated.  This  is  the 
sub -arachnoid  space  containing  cerebro-spinal  fluid. 

On  the  vertex  of  the  brain,  along  the  longitudinal  fissure,  there  are 
small  nodules,  outgrowths  of  the  arachnoid,  called  Pacchionian  bodies. 

Pia-mater. — It  is  an  extremely  thin  and  vascular  membrane  closely 


46 


THE  CENTRAL  NERVOUS  SYSTEM 


applied  to  the  cortex  and  continuing  in  the  fissures.     Together  with  the 
other  two  membranes  it  forms  the  sheaths  of  the  cranial  nerves. 

Special  processes  are  sent  off  in  the  spaces  between  certain  portions  of 
the  brain.  In  the  cavities  of  the  third  and  fourth  ventricles  they  are 
known  as  Tela  choroidea,  the  margins  of  which  are  wrinkled,  forming  a 
vascular  fringe  known  as  Choroid  plexus.  The  latter  is  also  found  in  the 
lateral  ventricles. 


Meningeal  branch  of 
anterior  ethmoidal  artery 


Meningeal  branch  of  pos- 
terior ethmoidal  artery 


Middle  meningeal 

artery 
OPHTHALMIC 
DIVISION  OF 
TRIGEMINUS 
THIRD  NERVE 
Cavernous  sinus 
TROCHLEAR  NERVE 
AUDITORY  AND 
FACIAL  NERVES 
Superior  petrosal  sinus 
Inferior  petrosal  sinus 
Petrosquamous  sinus 
SPINAL  ACCES- 
SORY NERVE 
Sigmoid  sinus 
Posterior  meningeal 
branch  of  vertebral 
artery 


Left  marginal  sinus 


Left  lateral  sinus 
Superior  sagittal  sinus 


Circular  sinus 
Carotid  artery 

ABDUCENS 
Basilar  artery 
Basilar  plexus  of  veins 
Auditory  artery 
Vertebral  artery 
j_  GLOSSOPHARYNGEAL 
I     AND  VAGUS  NERVES 
)    '  Anterior  spinal  artery 
HYPOGLOSSAL 

NERVE 
SPINAL  ACCES- 
SORY NERVE 

Right  marginal  sinus 

Occipital  si7ius 

Right  transverse  sinus 


Fig.  43.— Showing  Blood  Vessels  of  Cranial  Dura  Mater  and  Cranial  Nerves  in  the 
Base  of  the  Skull.     (Morris'  Anatomy.) 

(On  the  left  side  the  dura  mater  has  been  removed  in  the  middle  fossa.) 


BLOOD  SUPPLY  OF  THE  BRAIN 

The  arteries  of  the  brain  are  supplied  by  the  two  internal  carotids  and 
the  vertebrals. 

A.  The  internal  carotid  arteries  divide  into  anterior  and  middle 
cerebral  arteries  and  posterior  communicating. 

The  anterior  cerebral  arteries  supply  the  frontal  and  olfactory  lobes, 
the  optic  nerves,  corpus  callosum  and  anterior  perforated  space. 


THE  CENTRAL  NERVOUS  SYSTEM 


47 


Fig.  44. — Middle  Cerebral  Artery  and  Branches.     {Gordinier,  after  Quain  and  Charcot.) 

Cent.     Antero-lateral  group  of  ganglionic  arteries.     1.  Inferior   external   frontal   artery. 
2.  Ascending  frontal  artery.     3.  Ascending  parietal  artery.     4.  Parieto-temporal  artery. 


Anterior  cerebral 
artery 


Middle  cerebral 

artery 
Internal  carotid 

artery 
Poster  o-median 
perforating 
Posterior  cerebral 
artery 
Superior  cerebellar 
artery 

A  nlerior  inferior 
cerebellar  artery 


Vertebral  artery 


Posterior  inferior 
cerebellar  artery 


Anterior  com- 
municating 

artery 

Antero-lateral 
perforating 

Chorioid 

Posterior  com- 
municating 
artery 

Posterior  chorioid 

Basilar  artery 
Cerebellum,  cut 


Anterior  spinal 
artery 


Fig.  45. — The  Arteries  of  the  Brain.     {Morris'  Anatomy.) 
(The  cerebellum  has  been  cut  away  on  the  left  side  to  show  the  posterior  part  of  the  cere- 
brum.    From  a  preparation  in  the  Museum  of  St.  Bartholomew's  Hospital.) 


48 


THE  CENTRAL  NERVOUS  SYSTEM 


The  middle  cerebral  arteries,  the  largest  of  the  internal  carotid 
branches,  supply  the  frontal,  parietal  and  temporal  lobes,  and  through  the 
anterior  perforated  space  branches  to  the  basal  ganglia.  The  latter 
branches  are  the  " ganglionic."  One  of  the  lenticulo-caudate  arteries 
which  is  the  largest  is  called  "artery  of  cerebral  hemorrhage"  (Charcot). 

The  posterior  commirnicating  arteries  join  the  posterior  cerebral 
arteries. 

B.  The  Vertebral  arteries,  branches  of  the  subclavian,  meet  at  the 
lower  border  of  the  pons,  unite  and  form  one  trunk,  viz.  basilar  artery, 
which,  running  in  the  middle  line  of  the  pons,  divides  at  its  upper  border 
into  two  posterior  cerebral  arteries. 


Cortical  arteries 


External  striate  arteries 
Middle  cerebral  artery 


CA  UDA  TE  NUCLEUS 
THALAMUS 


TUBER  CINEREUM 


OPTIC  TRACT 


ANTERIOR  PERFORATED 
SUBSTANCE 


Internal  striate  arteries 

Fig.  46. — Diagram  showing  the  Manner  of  Distribution  of  the  Cortical  and  Central 
Branches  of  the  Cerebral  Arteries.     (Morris'  Anatomy.) 


The  basilar  artery  gives  off  anterior,  inferior  and  superior  cerebellar 
arteries.  At  the  base  of  the  brain  the  internal  carotids  and  the  vertebrals 
join  and  form  the  Circle  of  Willis  (see  illustration).  The  characteristic 
feature  of  the  blood  vessels  distributed  in  the  substance  of  the  brain  lies 
in  the  fact  that  they  are  terminal,  that  is,  they  do  not  anastomose  with 
each  other. 

Cerebral  veins  do  not  accompany  the  arteries,  but  open  in  various 
sinuses  (see  chapter  on  Meninges).  The  latter  are  without  valves  (Figs. 
44,  45  and  46). 


THE    CENTRAL   NERVOUS    SYSTEM  49 

HISTOLOGICAL  ELEMENTS  OF  THE  CENTRAL  NERVOUS  SYSTEM 

Neurone  Doctrine  and  Secondary  Degeneration 

The  chief  histological  elements  of  the  central  nervous  system  are: 
nerve-cells,  nerve-fibers,  neuroglia. 

Nerve-cell. — It  consists  of  a  protoplasmic  body  in  the  center  of  which 
is  a  nucleus  with  a  nucleolus.  It  has  no  capsule,  but  the  cells  of  the  sym- 
pathetic ganglia  and  of  the  spinal  ganglia  have  a  capsule.  The  proto- 
plasma  contains  granular  masses,  stainable  by  Nissl's  stain,  and  they  are 
called  Nissl's  bodies.  Ramon  y  Cajal  has  recently  (1903)  devised  a  stain 
with  which  it  can  be  demonstrated  that  the  cell  is  composed  of  delicate 
fibrils  (neurofibrils).  In  the  majority' of  the  cells,  particularly  in  those  of 
large  size,  exists  a  granular  mass  of  yellowish  pigment. 

Each  cell  is  provided  with  processes,  one  of  which  is  the  most  important 
and  called  axone. 

Varieties  of  Cells.— They  may  be  bipolar  and  multipolar. 

The  latter  are  found  throughout  the  entire  nervous  system,  but  pre- 
dominate in  the  anterior  horns  of  the  spinal  cord,  the  cortex,  medulla, 
basal  ganglia.  Bipolar  cells  are  found  in  the  posterior  horns  of  the  spinal 
cord,  spinal  ganglia,  and  in  the  molecular  layer  of  the  cerebellar  cortex. 

In  the  Cerebellum  there  are  two  special  forms  of  cells,  viz.  Purkinje's 
cells  and  basket  cells.  The  first  are  flask-shaped  with  a  very  slender  and 
long  axone  having  a  vertical  course.  The  second  have  also  a  long  axone 
which  has  a  horizontal  course. 

Cell  Processes. — Some  branches  of  a  cell  branch  out,  tree-like,  divide 
and  subdivide,  but  do  not  anastomose.  They  are  the  dendrites.  One 
process,  called  axone,  is  the  most  important.  It  gives  off  collaterals. 
Soon  after  leaving  the  cell  it  receives  a  coating,  called  myelin  and  becomes 
a  medullated  nerve-fiber. 

Nerve-fiber.- — It  is  the  prolongation  of  an  axone.  It  consists  of  an 
axone,  surrounded  by  a  myelin  sheath  and  of  a  delicate  membrane,  neuri- 
lemma, surrounding  the  latter.  The  myelin  and  neurilemma  present 
constrictions  (nodes  of  Ranvier). 

The  sympathetic  system  contains  nerve-fibers,  called  non-medullated, 
viz.  deprived  of  a  myelin  sheath. 

Connective  tissue  joins  nerve-fibers  into  fasciculi  or  bundles,  which 
when  united  form  peripheral  nerves. 

The  nerves  terminating  in  the  muscles  or  skin  present  at  their  ends 
special  arrangements.     The  motor  nerves   end  in  motorial  end  plates, 
which  are  special  expansions  of  granular  protoplasm.     The  sensory  nerves 
end  in  corpuscles,  composed  chiefly  of  connective  tissue. 
4 


5<D  THE   CENTRAL   NERVOUS    SYSTEM 

Neuroglia. — It  is  the  framework  of  the  central  nervous  system.  Be- 
fore Virchow  (1846)  it  was  considered  as  a  connective  tissue.  This 
tissue  is  composed  of  spheric  cells  (spider  cells,  Deiters'  cells)  containing 
a  large  nucleus  and  a  great  many  processes.  The  function  of  neuroglia 
tissue  is  to  replace  diseased  nervous  elements.  As  soon  as  a  lesion  of  the 
latter  occurs,  the  neuroglia  tissue  begins  to  proliferate.  Its  function 
therefore  resembles  that  of  connective  tissue. 

Neurone. — Under  this  name,  given  first  by  Forel  and  then  by  Wal- 
deyer,  is  understood  an  anatomical  unit  of  the  nervous  system.  It  con- 
sists of  the  cell-body  with  all  its  outgrowths,  viz.  dendrites  and  axone 
with  all  the  ramifications,  collaterals,  and  terminal  arborizations.  The 
nervous  system  is  made  up  of  a  multitude  of  such  units  which,  according 
to  the  generally  accepted  view,  are  trophically  and  genetically  independent 
and  are  only  contiguous,  but  not  continuous.  In  spite  of  their  independ- 
ence they  are  functionally  correlated.  This  can  be  explained  by  a  sup- 
posed amoeboid  function  of  the  dendrites  and  by  appearance  and  dis- 
appearance of  buds  or  gemmules  on  them  caused  by  the  contractility 
of  the  protoplasm.  Such  a  mechanism  will  naturally  bring  about  a  con- 
tact influence  of  one  neurone  upon  another.  The  problem,  however,  is 
far  from  being  settled. 

There  are  three  types  of  neurones: 

(1)  Neurone  with  a  long  axone  which  may  give  off  collaterals.  It 
is  found  in  brain  and  spinal  cord. 

(2)  Neurone  with  a  short  axone  which  breaks  up  promptly  into 
branches.     It  is  found  in  brain  and  cerebellum. 

(3)  Neurone  with  several  axones.  It  is  found  in  the  first  layer  of 
cerebral  cortex. 

The  function  of  neurones  is  (1)  efferent  (motor),  (2)  afferent  (sensory) 
and  (3)  associative. 

(1)  Efferent  (Motor)  Neurones. — They  present  two  groups:  upper  and 
lower. 

Upper  Motor  Neurones. — This  is  the  pyramidal  system.  The  fibers 
originate  in  the  large  pyramidal  cells  of  the  motor  area  (see  Localizations, 
page  75),  descend  through  the  centrum  ovale,  reach  the  internal  capsule, 
pass  downward  into  the  crura,  medulla  oblongata,  where  they  mostly 
decussate,  descend  in  the  spinal  cord  in  a  small  number  as  non-decussated 
or  direct  fibers  in  the  antero-internal  region  of  the  same  side,  and  in  a 
larger  number  as  decussated  or  crossed  on  the  opposite  side  as  low  as  the 
fourth  sacral  segment.  During  their  passage  through  the  mid-brain  and 
hind -brain  a  large  number  of  fibers  are  given  off  to  the  motor  nuclei  of 
the  cranial  nerves,  viz.  third,  sixth,  fifth,  seventh,  ninth  and  twelfth. 


THE  CENTRAL  NERVOUS  SYSTEM  5 1 

In  the  spinal  cord  the  crossed  pyramidal  fibers  give  off  collaterals  to  the 
gray  matters.  The  direct  pyramidal  fibers  decussate  through  the  anterior 
white  commissure  and  terminate  in  the  cornua  of  the  opposite  side. 
The  upper  motor  neurones  connect,  therefore,  the  motor  cortex  with  the 
bulbo-spinal  centers. 

Lower  Motor  Neurones. — They  are  constituted  by  the  motor  nuclei  of 
the  medulla  and  spinal  cord  (anterior  cornua),  their  roots  and  peripheral 
nerves.  In  the  medulla  the  nuclei  give  origin  to  the  motor  fibers  of 
cranial  nerves.  In  the  spinal  cord  the  cells  of  the  anterior  cornua  give 
origin  to  the  motor  nerves  of  the  muscles  of  the  extremities  and  the  body. 

(2)  Afferent  (Sensory)  Neurones. — They  commence  in  the  sensory 
end-organs,  ascend  in  the  peripheral  nerves  to  the  spinal  ganglia,  posterior 
roots  and  their  extension  into  the  spinal  cord  as  posterior  columns.  The 
latter  continue  their  course  through  the  mesial  fillet,  where  they  decussate 
and  reach  upward  the  optic  thalamus.  Another  set  of  fibers  originate 
in  the  cells  of  the  posterior  cornua,  decussate  in  the  posterior  commissure, 
reach  the  antero-lateral  tract  and  ascend  through  the  medulla  and  pons  to 
end  in  the  optic  thalamus  (spino-thalamic  system).  The  sensory  fibers  of 
the  cranial  nerves  also  enter  the  sensory  pathway.  Finally,  fibers  ascend 
from  the  optic  thalamus  to  the  cerebral  cortex  of  the  same  and  opposite 
side.  (For  details  of  the  pathways  see  their  respective  chapters  in 
Anatomy.) 

(3)  Association  Neurones. — They  connect  the  other  neurones  together. 
They  are  neurones  with  short  axones. 

The  integrity  of  a  neurone  depends  upon  the  integrity  of  its  main 
element,  viz.  cell-body.  Waller  has  shown  that  if  a  nerve  was  severed 
from  its  mother  cell,  it  would  degenerate  in  the  direction  in  which  the 
impulse  is  conducted.  For  example,  a  section  of  a  posterior  (sensory) 
nerve-root  ventrally  to  its  spinal  ganglion  in  which  it  originates,  will  be 
followed  by  a  degeneration  of  the  ascending  posterior  columns  of  the 
cord.  If  an  anterior  (motor)  root  will  be  severed  from  its  origin  in  the 
cells  of  the  anterior  cornua,  the  degeneration  of  the  peripheral  nerve  will 
be  descending  and  reach  its  termination  in  the  muscle.  This  process  is 
called  "secondary  degeneration." 

MALFORMATIONS  OF  THE  CENTRAL  NERVOUS  SYSTEM 

The  brain  and  spinal  cord  may  be  the  seat  of  malformations.  Patho- 
logical and  traumatic  influences  may  disturb  the  growth  of  the  foetus  in 
its  intra-uterine  life.  The  ealier  the  disturbances  occur,  the  more 
pronounced  are  the  malformations. 


52  THE  CENTRAL  NERVOUS  SYSTEM 

Brain. — Hyperplasia  or  Hypoplasia  of  cerebral  tissue  are  the  chief 
abnormalities. 

A.  Hypoplasia. — Absence  of  the  brain  is  called 

i.  Anencephalia.  (Fig  47.)  Instead  of  brain  tissue  there  is  a  cavity 
filled  with  a  dark  vascular  membrane.     This  condition  is  inconsistent 


Fig.  47. — Anencephalia. 

with  life.  Partial  absence  (agenesis)  of  brain  tissue  is  compatible  with 
life.  When  parts  of  the  cerebrum  are  not  properly  developed,  the  local 
defects  are  covered  with  the  pia-arachnoid;  the  subarachnoid  space  is 
tilled  with  fluid.     To  this  variety  belongs 

2.  Porencephaly.     (Fig.  48.)     It  is  characterized  by  a  fissure-like  or 
funnel-shaped  depression  showing  a  want  of  normal  tissue.     It  is  confined 


THE  CENTRAL  NERVOUS  SYSTEM 


53 


usually  to  the  cortex  (central  and  parietal  lobes),  and  the  Island  of  Reil, 
but  it  may  also  be  intracerebral.  The  depression  is  bridged  over  by  the 
arachnoid,  but  the  pia  follows  the  gyri.  It  may  reach  the  ventricles  and 
even  communicate  with  then.  Porencephaly  is  frequently  associated 
with  other  developmental  defects,  as  multiplication  of  fissures  and  lobes. 
Microscopically  there  is  atrophy  of  cells  and  white  matter  in  the  defective 
area.  Clinically  hemiplegia,  contractures,  imbecility,  idiocy,  mutism 
and  deafness  are  observed  (see  page  112).  Depressions  in  the  cerebrum 
are  sometimes  observed  when  partial  destruction  of  cortical  and  sub- 
cortical tissue  occurs  from  some  acquired  morbid  process  in  extra-  or  intra- 
uterine life.     This  is  pseudo-porencephaly. 

3.  Microcephalia  is  characterized  by  a  diminution  in  size  of  one  or 


Fig.  48. — Porencephaly.     (Feeble-mindedness.     Epilepsy.)     (Original.) 


both  hemispheres.  Normally  the  weight  of  a  male  brain  is  about  1375 
grm.  and  of  a  female  brain  1245  grm.  In  microcephalia  the  weight  may 
be  as  low  as  900  grm.  and  even  200  grm.  Either  the  entire  brain  or 
hemisphere  is  atrophied  or  there  is  a  defective  development  of  a  certain 
portion  of  them.  It  is  usually  due  to  a  defective  development.  It  may 
also  be  due  to  a  premature  closure  of  the  cranial  sutures.  There  is  often 
a  deficiency  of  the  sulci  on  the  cortex;  the  lobes  and  convolutions  are 
imperfectly  outlined  or  abnormally  small.  The  spinal  cord  is  also  very 
small;  the  postero-lateral  columns  are  mostly  affected.  Clinically  the 
condition  is  characterized  by  a  small  size  of  the  head  and  defective 
mental  development  (Fig.  49) . 

4.  Microgyria  consists  of  multiplication  of  fissures  and  consequently 


54 


THE    CENTRAL    NERVOUS    SYSTEM 


of  convolutions.     The  latter  are  very  small.     The  condition  is  not  in- 
frequently associated  with  microcephaly. 

5.  Absence  of  Lobes,  imperfect  development  or  absence  of  some  of 
the  basal  ganglia  and  of  Corpus  Callosum  also  occur  in  conjunction 
with  microcephaly. 

6.  The  Cerebellum  may  present  the  same  developmental  defects  as 
the  cerebrum.     Atrophy  of  the  cerebellum  was  the  subject  of  an  exhaustive 


Fig.  40 — Microcephaly.     (Idiot. 


study  by  Preisig  (/.  /.  Psych,  u.  Neurol.,  1911).  He  reaches  the  following 
conclusions:  (1)  There  is  a  form  of  meningo-encephalitis  of  fcetal  period 
of  life  with  a  special  localization  in  the  cerebellum  which  results  in  atrophy 
of  the  latter.  (2)  Idiocy  which  exists  in  such  cases  has  an  anatomical 
basis  in  conjunction  with  other  alterations  of  the  cerebral  cortex.  (3)  All 
the  systems  of  fibers  ending  in  the  cerebellum  are  in  a  state  of  atrophy. 
B.  Hyperplasia  is  characterized  by  an  excessive  development  of  the 


THE  CENTRAL  NERVOUS  SYSTEM 


55 


constituent  elements  of  the  brain.  Macrocephaly  is  due  to  hypertrophy 
of  the  brain.  There  is  anatomically  an  increase  of  gray  substance  and  of 
connective  tissue  in  the  white  matter.  The  skull  is  proportionally  in- 
creased and  the  head  appears  large. 

The  etiology  of  macrocephaly  is  alcoholism  and  syphilis  of  the 
parents.  Imbecility,  idiocy  and  convulsions  are  observed  in  macro- 
cephalic  individuals. 

Other  Malformations. — To  this  category  belong  cases  associated  with 
defects  in  the  development  of  the  cranium.  Encephalocele  is  character- 
ized by  a  protrusion  of  brain  tissue  between  the  cranial  bones,  which  for 


Fig.  50. — Spina  Bifida. 


some  reason  failed  to  coalesce.  If  the  meninges  alone  form  the  mass,  it 
is  called  Meningocele.     There  is  usually  a  combination  of  both  anomalies. 

Cyclopia  is  characterized  by  a  mass  in  the  forehead  consisting  of  fusion 
of  both  eyes.  When  in  addition  to  the  latter  there  is  also  absence  of  the 
nose,  the  anomaly  is  called  Arhinencephalia. 

Spinal  Cord. — The  most  common  variety  of  malformations  of  the 
spinal  cord  is  Spina  bifida.  It  is  due  to  a  defect  in  the  development  of  the 
vertebral  arches,  which  failed  to  coalesce  and  therefore  permitted  the  cord 
with  or  without  the  meninges  to  protrude  dorsally. 


50  THE  CENTRAL  NERVOUS  SYSTEM 

Spina  bifida  may  present  itself  as  a  Meningocele,  viz.  a  saccular  tumor 
containing  the  meninges;  as  a  Meningomyelocele,  consisting  of  meninges 
and  cord,  also  the  nerve-roots;  as  a  myelocele,  consisting  only  of  cord 
tissue;  the  latter  is  rare  and  occurs  when  not  only  the  vertebral  arches, 
but  also  the  medullary  folds  failed  to  coalesce. 


Fig.  51. — Meningomyclocele  with  Der- 
moid Cyst.     (Rigidity  of  all  extremities.) 


Fig.  52. — Meningomyclocele  with  Der- 
moid Cyst.     (Rigidity  of  all  extremities.) 


The  most  frequent  variety  is  the  Meningomyelocele.  Clinically  there 
is  usually  paralysis  of  the  legs,  of  bladder  and  rectum,  clubfoot,  also 
anaesthesia.  The  lumbo-sacral  region  is  its  place  of  predilection  (Fig.  50, 
51,  52)- 


THE    CENTRAL    NERVOUS    SYSTK.M 


57 


Children  born  with  a  spina  bifida  usually  do  not  live  long;  their  average 
life  is  from  three  months  to  a  year.  In  some  cases  where  the  tumor  is 
very  small,  adult  life  may  be  reached.  The  covering  of  the  tumor  may  be 
only  a  thin  membrane  or  else  the  skin.  In  the  latter  case  the  prognosis 
to  life  is  better,  as  the  skin  is  a  good  protection. 

The  tumor  may  be  a  round  mass  covered  by  and  continuous  with  the 
surrounding  skin  or  may  be  pedunculated.  In  the  latter  case  there  is 
usually  a  meningocele.  Operative  procedures  are  practically  the  only 
means  that  may  be  employed  for  remedying  the  condition.  In  the  most 
favorable  cases  paralysis  of  the  extremities  will  remain.     In  meningo- 


FiG.  53. — Diplomyelia.  (Original.) 


myelocele  the  contents  of  the  sac  cannot  be  removed.  Only  in  pure 
meningocele  an  operation  can  give  satisfactory  results.  Tournier  and 
Ducuing  basing  themselves  on  a  large  experience  formulate  the  following 
indications  for  operative  treatment.  Excision  of  the  tumor  is  permissible 
when  it  is  complicated  with  nervous  symptoms  of  no  longer  than  a  year's 
duration  at  most,  or  when  the  tumor  is  free  from  complications,  but  is  of 
annoying  size.  When  the  nervous  symptoms  are  of  long  duration, 
palliative  treatment  is  the  rule,  some  orthopedic  operation  on  other  parts 
of  the  body  than  on  the  spina  bifida  offering  the  only  prospect  of  benefit. 
Under  all  other  conditions  operative  treatment  is  contraindicated  (Arch, 
gener.  de  Chir.,  1912). 

Among  other  malformations  can  be  mentioned:  Craniorrhachischisis 


58  THE  CENTRAL  NERVOUS  SYSTEM 

and  Rhachischisis.  The  first  consists  of  lack  of  closure  of  the  cranium 
and  vertebrae.  The  entire  medullary  groove  is  patent  and  filled  with  a 
vascular  tissue,  rudiments  of  cord  and  brain. 

In  the  second  variety  only  the  spinal  portion  of  the  groove  is  patent. 

As  a  great  rarity  may  be  mentioned  diplomyelia  (double  cord) 
(Fig.  53)- 

Heterotopia  of  gray  matter  is  also  a  very  rare  condition.  It  consists 
of  the  presence  of  gray  nodules  in  the  white  matter  of  the  cord. 


CHAPTER   II 

METHOD  OF  EXAMINATION  FOR  DIAGNOSIS  OF 
NERVOUS  DISEASES 

The  diagnosis  of  cases  of  nervous  diseases  is  based  chiefly  upon  the 
knowledge  of  the  physiological  and  pathological  states  of  the  following 
elements:  I,  motor  phenomena;  II,  sensory  phenomena;  III,  special 
senses ;  IV,  cranial  nerves ;  V,  speech  and  writing ;  VI,  reflexes ;  VII, 
sphincters ;  VIII,  vasomotor  and  trophic  disturbances. 

In  order  to  arrive  at  a  correct  diagnosis,  every  case  of  functional  or 
organic  nervous  diseases  that  comes  under  observation  should  be  investi- 
gated from  the  above  standpoints. 

I.  MOTOR  PHENOMENA 

An  examination  of  the  motor  phenomena  embraces  the  following 
points:  station,  gait,  attitude,  muscular  power,  various  muscular  move- 
ments, state  of  nutrition  of  muscles. 

Station. — The  patient  is  observed  while  standing  with  open  and  closed 
eyes.  Normally  when  the  eyes  are  closed  and  the  patient  stands  with  his 
feet  close  together,  there  is  a  very  slight  oscillation  of  the  body.  When 
the  latter  is  somewhat  more  than  slight  or  very  pronounced,  so  that  the 
patient  shows  a  tendency  of  falling,  the  station  is  pathological.  In  neur- 
asthenia, where  the  muscular  fatigue  is  pronounced,  an  instability  may  be 
present.  The  degree  of  the  latter  depends  upon  the  intensity  of  fatigue. 
It  never,  however,  reaches  the  instability  of  organic  nervous  diseases.  In 
hysteria  the  restlessness  and  inability  of  controlling  the  latter  may  also 
render  the  station  with  closed  eyes  abnormal. 

In  tabes  the  patient  cannot  hold  himself  straight  not  only  with  closed 
but  also  with  open  eyes.  In  the  latter  case  he  stands  with  the  feet  wide 
apart  and  the  least  attempt  to  bring  them  together  makes  him  lose  the 
equilibrium.  When  the  eyes  are  closed  the  instability  is  great  (Romberg's 
sign). 

The  same  condition  may  be  encountered  in  other  organic  nervous 
diseases.  Whether  it  is  due  to  organic  or  functional  nervous  diseases 
an  inability  or  difficulty  of  standing  erect  with  open  or  closed  eyes  is 
a  morbid  phenomenon. 

59 


60  EXAMINATION   FOR   NERVOUS    DISEASES 

Gait. — Disturbance  of  gait  is  very  frequently  observed  in  nervous 
diseases.  The  patient  is  told  to  walk  with  open  and  closed  eyes.  Nor- 
mally the  body  follows  a  straight  line.  In  certain  nervous  affections  the 
coordination  of  movements  is  disturbed.  This  is  called  ataxia.  The 
latter  may  be  very  slight,  moderate  or  much  pronounced.  It  is  par- 
ticularly marked  when  the  eyes  are  closed  or  when  the  patient  is  told  to 
turn.  In  ataxic  gait  the  patient  throws  his  leg  forward  in  an  exaggerated 
manner;  the  moment  he  is  ready  to  touch  the  ground,  the  foot  makes  a 
backward  movement  and  strikes  the  ground  with  the  heel.  Such  a  gait 
is  seen  in  tabes.  In  some  cases  the  gait  may  simulate  a  zigzag  movement. 
It  is  observed  particularly  in  cerebellar  diseases  (titubation) . 

Ataxia  may  also  be  revealed  by  asking  the  patient  to  touch  with  the 
heel  of  one  limb  the  knee  of  the  other  or  place  one  foot  on  the  other.  He 
will  then  form  circles  in  the  air  with  the  foot  before  it  reaches  the  desired 
spot. 

Besides  an  ataxic  gait,  there  is  also  a  high  steppage  gait,  which  is  due 
to  foot-drop.  The  thighs  are  flexed  on  the  abdomen  more  abruptly  and 
higher  than  normally;  the  foot  is  thrown  forward  and  then  it  falls  on  the 
ground  first  by  the  toes  and  last  by  the  heel,  so  that  two  successive  knocks 
on  the  ground  are  heard. 

Wh  ether  the  incoordination  is  mild  or  great,  it  is  a  morbid  phenomenon 
and  may  be  encountered  in  organic  and  in  functional  nervous  diseases. 
In  hysteria  there  may  be  an  inability  to  stand  or  walk  while  all  the  other 
functions  of  the  limbs  are  intact  when  the  patient  is  in  bed .  The  condition 
is  known  under  the  name  of  Astasia-abasia.  In  Paralysis  Agitans  the 
patient,  once  started  voluntarily  or  slightly  pushed,  may  keep  on  walking 
or  running  forward,  backward  or  laterally  for  a  long  while  until  stopped. 
These  movements  are  called,  respectively,  propulsion,  retropulsion  or 
lateropulsion. 

Ataxia  may  be  also  observed  in  the  upper  extremities.  The  patient 
is  told  to  close  his  eyes  and  bring  the  tips  of  the  fingers  of  both  hands 
together,  or  to  touch  the  ear  or  the  end  of  the  nose  with  one  finger.  This 
test  will  reveal  whether  the  movements  of  the  upper  limbs  are  coordinate 
or  not. 

In  connection  with  the  gait  the  attitude  of  the  patient  must  be  noticed, 
viz.  whether  he  holds  himself  in  an  erect  position  or  else  in  inclined 
forward  or  backward,  also  whether  the  movements  of  the  spinal  vertebra? 
are  free  or  not.  Special  attitudes  are  acquired  in  some  functional  and 
organic  nervous  diseases. 

Muscular  Power. — It  may  be  impaired  from  a  simple  weakness  to  a 
complete  loss.     When  a  muscle  is  suspected  to  be  diseased,  its  power  can 


EXAMINATION   FOR   NERVOUS   DISEASES  6 1 

be  tested  by  the  resistance  method.  The  patient  is  told  to  perform  the 
function  of  this  muscle  and  the  observer  endeavors  to  resist  the  given 
movement.  If,  for  example,  the  forearm  is  voluntarily  flexed  (biceps), 
an  effort  is  made  to  extent  it.  When  the  resistance  of  the  biceps  is 
easily  overcome,  we  say  that  there  is  diminution  of  power  of  that  muscle. 
If  the  desired  function  of  a  certain  muscle  cannot  be  performed  at  all, 
it  may  be  due  either  to  paralysis  or  atrophy  of  that  muscle. 

When  paralysis  is  present,  it  may  be  complete  or  incomplete  (paresis). 
It  may  be  spastic  when  associated  with  rigidity  of  the  muscles  (hypertonia) 
and  flaccid  when  the  muscles  are  flaccid  (hypotonia).  The  spastic  form 
can  be  revealed  by  the  presence  of  enormous  resistance  of  the  muscles  to 
passive  movements.  The  flaccid  form  is  detected  from  the  total  absence 
of  resistance.  In  the  latter  case  if  the  hypotonia  is  pronounced,  the  limbs 
may  be  made  to  assume  unusually  abnormal  positions,  such  as  raising  the 
foot  to  the  head. 

Paralysis  may  affect  one  limb  (monoplegia),  two  symmetrical  limbs 
(paraplegia)  or  one  side  of  the  body  (hemiplegia).  When  the  four  ex- 
tremities are  affected  with  a  spastic  paralysis,  we  speak  of  diplegia. 
Paralysis  or  paresis  may  affect  the  musculature  of  an  entire  limb  or  only  a 
certain  group  of  muscles,  as,  for  example,  in  cases  of  wrist-  or  foot-drop? 
in  which  the  extensor  groups  of  muscles  alone  are  affected. 

Muscular  Movements.- — The  manner  of  using  the  lower  extremities, 
of  grasping  or  handling  objects  or  else  doing  certain  acts  with  the  upper 
extremities,  the  range,  rhythm,  regularity  or  irregularity,  constancy  or 
inconstancy  of  the  movements,  the  effect  of  emotion  on  movements, 
must  all  be  closely  observed  in  studying  the  motor  apparatus.  Ataxia, 
paralysis,  loss  of  muscular  resistance  were  mentioned  above.  Tremor  is 
observed  in  functional  and  organic  nervous  diseases.  It  may  be  fine, 
coarse,  passive  (when  at  rest)  and  intentional  (upon  voluntary  act). 
It  may  be  also  temporary  or  permanent.  Choreic  movements  consist 
of  irregular,  involuntary  and  continuous  muscular  contractions. 

Athetosis    consists   of    slow    and    arhythmical   movements,    affecting 
chiefly  the  fingers. 

Tic  is  a  quick  involuntary  contraction  of  a  certain  muscle  or  group  of 
muscles  continuously  repeating  itself  in  a  regular  manner  and  imitating 
ordinary  mimicry. 

Myoclonia  is  characterized  bv  involuntary  clonic  contractions  of  a 
group  of  muscles  occurring  in  paroxysms.  Tetany  consists  of  a  sudden 
and  special  contraction  of  the  muscles  of  the  fingers;  they  assume  a  writing 
position. 

Muscles  may  also  be  affected  by  convulsive  movements,  spasms  or 


62 


EXAMINATION   FOR   NERVOUS   DISEASES 


cramps.     All  these  muscular  phenomena  occur  either  independently  of  or 
in  association  with  various  organic  or  functional  nervous  diseases. 

State  of  Nutrition  of  the  Muscles. — The  nutrition  of  the  muscles 
depends  upon  the  integrity  of-  their  trophic  centers.  When  a  muscle  is 
diminished  in  volume  and  cannot  therefore  perform  its  function,  we 
speak  of  its  Atrophy.  When  the  atrophy  is  symmetrical  and  slight,  it 
may  escape  our  notice.  When  it  is  unilateral,  it  will  be  revealed  by 
comparison  with  the  corresponding  muscles  of  the  opposite  side. 


Frontalis 

Facial  {upper) 

Corrugator    supercilii 

Orbic.    palpebrarum 
Nasal  muscles 

Zygomatici 
Orbic.     oris 

Facial  (middle) 
Masseter 

Levator  menti 

Quadratus 

Triangularis 

Hypoglossus 

Facial  (lower) 

Platysma  myoides 

Hyoid  muscles.  \ 


Omohyoid 


Ext.    anterior    thoracic 
(pectoralis  major). 


Ascending  frontal  and 
parietal  convolutions 
(motor  area) . 


Third  frontal  convolu- 
tion and  insula  (cen- 
ter of  speech). 

Temporalis. 

Facial  (upper  branch). 
Facial  (trunk). 

Posterior  auricular. 

Facial  (middle  branch) . 
Facial  (lower  branch). 
Splenius. 

Sternomast  oi  deus . 
Spinal  accessory 
Levator  anguli  scapulae 
Trapezius. 

D  or s ali s  scapula 
(rhomboid  ei). 

Circumflex. 

Long  thoracic  serratus 
magnus) . 


Phrenic.  Fifth  and  sixth  cervical 
(deltoid,  biceps,  bra- 
chialis  anticus,  supi- 
nator longus). 


Brachial  plexus. 


Fig.  54. — Motor  Points  of  Face  and  Neck. 


It  is  not  sufficient  to  determine  the  size  of  muscles.  It  is  also  necessary 
to  study  the  state  of  their  functions:  a  diminution  of  muscular  force  will  be 
a  valuable  information  in  case  the  reduction  of  the  volume  of  the  muscle 
is  not  perceptible.  If  the  size  of  a  muscle  is  increased,  we  speak  of 
Hypertrophy.  The  size  of  a  muscle  or  of  a  group  of  muscles  may  be  only 
apparently  increased  when  the  atrophy  is  masked  by  an  increase  of  su- 
perimposed adipose  tissue.  Such  a  condition  is  known  under  the  name 
of  Pseudo -hypertrophy.     Finally  in  cases  where  inspection  or  palpation 


EXAMINATION   FOR   NERVOUS   DISEASES 


63 


make  the  diagnosis  of  atrophy  doubtful,  an  electrical  examination  must 
be  resorted  to. 

Electrical  Contractility. — Faradism  and  galvanism  are  employed  for 
the  purpose  of  ascertaining  the  state  of  nutrition  of  muscular  tissue  and 
of  the  nerves  distributed  in  them.     Muscular  contractions  may  be  ob- 


Deltoid  (posterior  part)' 


Musculospiral, 
Brachialis  anticus. 

Supinator  longus. 
Ext.  carpi  radial,  longior. 
Ext.  carpi  radial,  brevior. 


Extensor  communis 
digitorum. 


Extensor  indicis 


Ext.  ossis  metacarpi  pollicis. 
Ext.  primi  internodii  pollicis. 


Dorsal  interossei.  \  tt' 


Triceps  (long  head). 


Triceps  (outer  head). 


Extensor  carpi  ulnaris. 
Supinator  brevis. 

Extensor  minimi  digiti. 
Extensor  indicis. 

Extensor     secundi    internodii 
pollicis. 


Abductor  minimi  digiti. 
Dorsal  interossei  (III  and  IV). 


Fig.  55. — Motor  Points  on  Upper  Limb,  Extensor  Surface. 


tained  from  direct  applications  of  the  electrical  current  to  the  tested 
muscle  or  from  its  application  to  the  nerve  trunks. 

The  examination  for  electrical  reactions  must  be  conducted  in  the 
following  manner. 

Faradism. — The  large  or  indifferent  electrode  attached  to  the  positive 


64 


EXAMINATION    FOR    NERVOUS    DISEASES 


pole  (anode)  is  applied  to  the  neck  or  sternum,  the  small  or  testing  elec- 
trode attached  to  the  negative  pole  (cathode)  is  held  in  the  operator's 
hand.     Both  electrodes  must  be  well  wetted  in  hot  water. 


Triceps  (long  head) 


Triceps  (inner  head) 


Ulnar 


Flexor  carpi  ulnaris. 

Flexor  profundus 
digitorum. 


Flexor    sublimis    digi- 
torum (II  and  III). 


Flexor    sublimis    digi-, 
torum      (index     and 

little  fingers). 

Ulnar. 

Palmaris  brevis. 

Abductor  min.  digit. 

Flexor  min.  digit. 

Oppenens  min.  digit. 


Lumbricales.  \ 


•  Median. 
Supinator  longus. 

Pronator  teres. 


Deltoid      (anterior 
portion) . 


Musculocutaneous. 

Biceps. 

Brachialis  anticus. 


Flexor  carpi  radialis. 

Flexor  sublimis  digitorum. 

Flexor  longus  pollicis. 
Median. 

Abductor  pollicis. 
Opponens  pollicis. 

Flexor  brevis  pollicis. 

Adductor  pollicis. 


Fig.  56. — Motor  Points  on  Upper  Limb,  Flexor  Surface. 

The  current  is  slowly  turned  on  and  the  contact  breaker  of  the 
battery  having  been  put  to  its  slowest  rate  of  vibration,  the  testing  elec- 
trode is  placed  over  the  motor  point  of  the  muscle  or  on  the  nerve  trunk. 


EXAMINATION   FOR   NERVOUS   DISEASES 


65 


Normally  a  muscular  contraction  from  a  faradic  current  is  short,  stronger 
in  opening  than  in  closing  the  current.  If  the  current  is  rapid  and  follows 
one  another,  a  tetanic  contraction  is  obtained. 

Galvanism. — The  electrodes  are  placed  like  in  faradism.  The  testing 
electrode  is  attached  to  an  interrupting  handle  and  placed  upon  a  motor 
point.  At  each  closure  a  short,  sharp  muscular  contraction  appears. 
The  galvanometer  will  register  the  intensity  of  the  current.  Contractions 
are  obtained  in  cathodic  closure  (CaCC),  cathodic  opening  (CaOC), 
anodic  closure   (AnCC),   and   anodic  opening   (AnOC)    operations.     In 


Anterior  crural 


Adductor  magnus. 
Adductor  longus. 


Tensor  fasciae  f emoris. 


— -  Sartorius. 


—  Quadriceps  femoris. 


Rectus  femoris. 


Vastus  externus. 


Vastus  internus  J 


Fig.  57. — Motor  Points  on  Thigh,  Anterior  Surface. 


health  nerves  and  muscles  react  differently  whether  the  testing  electrode 
is  connected  with  the  Ca  or  An  and  whether  the  current  is  closed  or  opened. 
The  following  law  may  be  formulated  for  the  nerves:  (1)  with  a  weak 
current  a  reaction  is  obtained  only  from  CaCC;  (2)  with  a  medium 
current  the  reaction  from  CaCC  is  the  strongest,  although  anodal  contrac- 
tions are  also  obtained;  (3)  with  a  strong  current  the  CaCC  is  tetanic, 
iVnOC  and  AnCC  is  strong,  especially  the  first,  and  CaOC  is  weak.  In 
s 


66 


EXAMINATION   FOR   NERVOUS    DISEASES 


direct  muscular  contractions  only  the  closure  contractions  are  important. 
The  CaCC  comes  before  the  AnCC. 

Pathological  Reactions. — In  diseased  conditions  of  the  muscles 
quantitative  and  qualitative  changes  of  the  electrical  reactions  are  impor- 
tant. As  to  the  first  they  are  of  less  value  than  the  second.  An  increased 
reaction  has  an  importance  only  in  one  disease,  viz.  tetany.  There  the 
CaCC  and  AnOC  appears  even  with  very  weak  currents.  A  decreased 
response  occurs  oftener  than  an  increased,  but  without  any  special  diag- 
nostic value.     Ordinary  diminution  of  electric  irritability  is  observed  in 


Tibialis  anticus.  — 
Extensor  longus  digitorum 


Peroneus  brevis. — 


Extensor  longus  hallucis 


Interossei. 


Peroneal. 


Gastrocnemius. 
Peroneus  longus. 


Soleus. 


Flexor  longus  hallucis. 


Extensor  brevis  digitorum. 
Abductor  minimi  digiti. 


Fig.  58. — Motor  Points  on  Leg,  External  Surface. 

muscular  atrophy  without  degeneration  of  nerves  and  muscles,  in  muscular 
wasting  accompanying  diseases  of  the  joints  or  cerebral  diseases.  Com- 
plete loss  of  electrical  contractility  is  observed,  when. the  muscles  are  en- 
tirely destroyed.  Of  considerably  greater  importance  are  the  qualitative 
changes,  viz.  reactions  of  degeneration  (RD).  They  may  be  complete 
or  partial. 

Complete. — 1.     If  a  nerve  is  gravely  injured,  we  have  in  the  per- 
ipheral portion  an  increased  reaction  during  the  first  two   days  which 


EXAMINATION   FOR    NERVOUS    DISEASES 


67 


gradually  decreases  and  in  ten  to  fourteen  days  disappears  totally.     (The 
reactions  are  identical  with  galvanic  and  faradic  currents.) 

2.  In  case  of  a  muscle  being  gravely  damaged  the  galvanic  irritability 
decreases  first,  but  in  a  couple  of  weeks  increases  and  becomes  exaggerated. 
The  quickness  of  each  individual  response  is  lost  and  the  contractions 
become  sluggish.  At  the  same  time  there  is  a  change  in  the  normal  law 
of  contractions  (see  above),  viz.  the  AnCC  is  equal  to  or  stronger  than  the 


Sciatic. 

Biceps  (long  head). 
Biceps  (short  head). 


Peroneal. 


Gastrocnemius  » 
(outer  head). 


Soleus. 


Flexor  longus  hallucis. 


Gluteus  maxim  1 


^  j —  Adductor  magnus. 
■j —  Semitendinosus. 
I Semimembranosus. 


I Posterior  tibial. 


Gastrocnemius 
(inner  head) . 


Soleus. 


Flexor  longus  digitorum. 


Posterior  tibial. 


Fig.  59. — Motor  Points  on  Lower  Limb,  Posterior  Surface. 

CaCC  (reversed  formula).  Then  appear  the  CaOC  and  AnOC,  which 
normally  do  not  exist.  The  increased  galvanic  irritability  exists  for  one 
or  two  months,  then  gradually  disappears,  while  the  RD  persists.  In 
incurable  cases  the  galvanic  change  grows  deeper  and  deeper,  has  yet 
a  weak  AnCC  and  finally  the  latter  disappears.  As  to  the  faradic  con- 
tractility of  muscles  in  complete  RD,  it  is  lost. 


68  EXAMINATION    FOR    NERVOUS   DISEASES 

Partial  RD. — Here  the  change  in  the  galvanic  muscular  reactions 
is  the  same  as  in  complete  RD,  but  there  is  no  loss  of  galvanic  nerve  re- 
action and  no  loss  of  faradic  muscle  and  nerve  reactions. 

H.  SENSORY  PHENOMENA 

The  study  of  sensory  disturbances  constitutes  a  very  important  chap- 
ter in  diagnosis  of  nervous  diseases. 

Sensations  are:  general  and  special. 

The  special  concern  sight,  hearing,  smell  and  taste. 

The  general  sensations  are  superficial  and  deep.  To  the  first  belong: 
touch,  pain  and  temperature.  The  second  constitute:  muscular  and 
osseous  senses,  vibration  of  a  tuning  fork,  position,  pressure,  localization, 
recognition  of  two  points  of  compass,  also  stereognostic  sense. 

General  sensations  may  be  subjective  and  objective. 

Subjective. — They  are  various  sensations  felt  spontaneously  and  with- 
out any  external  stimulation.  Pain  may  be  continuous  or  paroxysmal, 
dull  or  sharp,  shooting,  lancinating.  Besides  pain  there  is  a  great  variety 
of  peculiar  sensations  called  paresthesia.  The  most  common  among  them 
are:  numbness,  tingling,  vibration,  affecting  especially  the  extremities. 
Sometimes  there  is  a  sensation  of  heat,  cold,  pins  and  needles  or  electricity. 
They  are  encountered  in  the  course  of  various  functional  and  organic 
nervous  diseases.     Not  infrequently  they  precede  the  onset  of  a  disease. 

Pain  is  a  frequent  symptom  and  sometimes  its  seat,  the  character  and 
the  mode  of  appearance  play  an  important  part  in  the  diagnosis  of  nervous 
affections. 

Objective  Sensations.  Superficial. — The  sense  of  touch  is  a  very 
important  element  in  diseases  of  the  nervous  system.  It  may  be  dimin- 
ished (hypaesthesia),  lost  (anaesthesia)  or  exaggerated  (hyperesthesia) . 
The  examination  for  touch  must  be  conducted  in  a  very  careful  manner. 
With  a  piece  of  cotton-wool,  or  with  von  Frey's  hairs  or  with  the  finger 
or  with  an  aesthesiometer  various  portions  of  the  body  are  touched  very 
superficially  and  then  more  closely.  The  promptness  of  response  is 
noticed.  If  one  portion  of  the  body  presents  changes,  it  is  advisable  for 
comparison  to  perform  the  same  manipulations  on  the  corresponding  part 
of  the  opposite  side. 

The  same  rules  can  be  applied  to  the  tests  for  temperature  (heat  and 
cold)  and  pain.  For  temperature  two  glass  tubes  half  filled  with  very 
hot  and  very  cold  water  are  used.  For  pain  the  sharp  end  of  a  pin  or 
aesthesiometer  is  used.  The  three  senses  may  all  be  abolished,  diminished 
or  exaggerated.     They  may  be  dissociated  so  that  touch  is  preserved, 


EXAMINATION    FOR    NERVOUS    DISEASES  69 

pain  and  temperature  are  abolished.  One  of  the  three  may  be  altered  to 
a  certain  extent.  The  heat  and  cold  may  be  reversed,  viz.  application 
of  heat  will  give  the  sense  of  cold  or  vice  versa.  Sensory  dissociation  is 
characteristic  of  syringomyelia,  but  may  be  observed  also  in  myelitis, 
hematomyelia,  Brown-Sequard's  syndrome. 

They  may  be  altered  on  a  half  of  a  body,  on  a  portion  of  it  or  on  a 
segment  of  a  limb.  Sensory  disturbances  may  follow  the  course  of  nerve- 
trunks  (radicular  anaesthesia).  They  may  be  distributed  in  islets  on 
various  portions  of  the  body  or  in  segments  the  upper  border  of  which 
is  perpendicular  to  the  axis  of  the  limb  (glove-like,  stocking-like).  Under 
the  name  of  Allochiria  is  understood  an  inability  to  recognize  the  spot 
touched  or  otherwise  stimulated  or  else  the  patient  points  to  the  side 
opposite  to  the  one  touched,  as  for  example  when  one  leg  is  touched  he 
thinks  that  it  was  the  other  leg.  It  is  met  with  in  hysteria  and  in  some 
organic  nervous  diseases. 

Deep  Sensations.  Pressure. — Various  weights  are  used  for  this  pur- 
pose. The  superficial  senses  may  be  abolished,  but  sense  of  pressure 
may  be  preserved. 

Localization. — It  can  be  determined  by  three  procedures:  (i)  naming, 
which  consists  of  having  the  patient  tell  the  spot  touched  while  his  eyes 
are  closed;  (2)  looking,  which  consists  of  pointing  out  the  touched  spot 
by  the  patient  (eyes  closed);  (3)  spacing,  which  consists  of  touching  with 
two  points  of  the  compass  or  with  one  point  after  another.  The  distance 
between  the  points  should  be  noticed. 

Muscular  Sense  and  Sense  of  Attitude. — The  patient  is  told  to  exe- 
cute certain  movements,  the  eyes  being  closed.  If  errors  are  made,  the 
muscular  sense  is  disturbed.  It  is  seen  particularly  in  tabes.  The  sense 
of  attitude  can  be  tested  by  placing  a  limb  or  a  portion  of  it  in  certain 
positions  and  by  asking  the  patient  with  the  eyes  closed  to  indicate  the 
position. 

Osseous  Sensation. — It  is  tested  with  a  tuning  fork.  The  latter  is 
applied  to  various  accessible  portions  of  the  skeleton.  A  sensation  of 
trepidation  is  obtained.  It  may  be  present  in  spite  of  cutaneous  anaes- 
thesia.    It  may  be  absent  in  tabes,  syringomyelia,  hematomyelia  (Egger). 

Stereognostic  Sense. — It  is  the  faculty  of  recognizing  by  palpation  the 
form,  shape,  consistency  of  objects,  also  the  material  of  which  they  are 
made.  The  patient  is  told  to  close  his  eyes  and  the  objects  are  placed 
in  his  hand.  The  loss  of  this  faculty  is  known  under  the  name  of  Aster  - 
eognosis. 

Asymbolia  (or  tactile  agnosia)  is  loss  of  ability  to  recognize  by  pal- 
pation the  nature  of  an  object  and  therefore  to  name  it.     Astereognosis 


70  EXAMINATION   FOR    NERVOUS    DISEASES 

and  asymbolia  may  exist  independently  of  each  other  and  each  of  these 
two  senses  may  be  present  while  all  other  sensibilities,  superficial  or  deep, 
are  absent.  (See  my  contributions  in  Med.  Record,  1910,  and  /.  of  Ab- 
normal Psychology,  191 1.) 

Head  proposes  the  following  grouping  of  sensations: 

1.  Epicritic  sensibility,  to  which  belong:  light  touch,  light  temperature 
of  heat  and  cold,  appreciation  of  two  points  of  compass  and  localization 
on  the  skin. 

2.  Protopathic  sensibility  to  which  belong:  extreme  temperature  of 
heat  and  cold,  appreciation  of  pain  sense  and  sensibility  of  the  viscera. 

3.  Deep  sensibility  to  which  belong:  pressure,  position,  movement. 
Experimenting  on  himself  Head  ascertained  that  section  of  a  peripheral 

nerve  produces  a  loss  of  protopathic  sensibility  over  the  area  of  distri- 
bution of  this  nerve  and  a  loss  of  epicritic  sensibility  over  a  larger  area. 
If  the  nerve  contains  sensory  and  motor  fibers,  deep  sensibility  is  also 
abolished.  When  a  nerve  is  only  slightly  injured,  the  epicritic  sensibility 
alone  is  impaired.  When  a  nerve-root  is  involved,  the  protopathic  sen- 
sibility is  more  impaired  than  the  epicritic.  The  nearer  the  lesion  lies  to 
the  central  nervous  system,  the  more  definite  and  more  extensive  is  the 
loss  to  pain ;  the  nearer  to  the  periphery,  the  greater  is  the  loss  to  cuta- 
neous touch.  In  this  way  a  diagnosis  of  the  position  of  the  lesion  can  be 
made. 

IE.  SPECIAL  SENSES 

Sight. — Changes  in  the  eyes,  especially  in  the  fundi,  are  of  great 
diagnostic  importance.  Optic  neuritis,  optic  atrophy,  swelling  of  the 
papilla  are  frequent  phenomena  of  cerebral  or  spinal  lesions.  The  ex- 
amination should  also  include  the  state  of  the  pupils,  their  light  and 
accommodation  reflexes,  their  shape,  their  relative  size. 

The  condition  of  the  ocular  muscles  should  be  ascertained.  Contrac- 
tion of  the  visual  and  color  fields  is  of  importance.  The  visual  acuity, 
partial  or  complete  blindness,  hemianopsia — all  these  symptoms  present 
valuable  data  for  diagnosis.  Subjective  visual  phenomena  (sparks, 
etc.)  are  not  infrequent  in  functional  nervous  diseases  (for  details  see 
Diseases  of  the  Second  Cranial  Nerve). 

Hearing. — Subjective  auditory  symptoms  are  of  great  importance. 
The  acuity  of  hearing  should  be  investigated,  either  with  a  watch,  tuning 
fork  or  whispered  voice.  There  may  be  also  perverted  hearing  (for  de- 
tails see  Diseases  of  the  Eighth  Nerve). 

Smell. — There  may  be  a  diminution,  increase  or  loss  of  sense  of  smell. 


EXAMINATION   FOR    NERVOUS    DISEASES  7 1 

A  perverted  smell  also  occurs.     Smell  can  be  tested  by  means  of  various 
odorous  substances. 

Taste. — It  should  be  investigated  with  various  substances  and  elec- 
trical current.     It  may  be  perverted  or  lost  in  nervous  affections. 

IV.  CRANIAL  NERVES 

The  cranial  nerves  are  very  frequently  involved  in  organic  diseases 
of  the  nervous  system.  Every  case  should  be  investigated  from  this 
standpoint.  Sometimes  in  doubtful  cases  the  pathological  condition  of 
a  cranial  nerve  decides  the  diagnosis  and  the  localization  of  the  lesion 
(see  Diseases  of  the  Cranial  Nerves). 

V.  SPEECH  AND  WRITING 

Disturbances  of  the  speech  are  quite  frequent  in  cerebral  diseases  of 
a  localized  nature,  in  some  affections  of  the  spinal  cord  and  sometimes  in 
hysteria. 

The  function  of  speech  should  be  investigated  from  the  following 
points  of  view:  articulation  of  words,  intonation,  reading,  writing,  copying, 
counting,  singing,  hearing  spoken  words.  A  disturbance  of  each  of  these 
faculties  has  a  special  significance.  See  chapter  on  Aphasia  and 
Agraphia. 

A  tremulous  speech,  a  certain  manner  of  pronouncing  certain  letters 
or  syllables  are  met  in  some  organic  affections  of  the  spinal  cord  and  in 
neuroses.  A  careful  examination  of  the  speech  is  necessary  and  any 
deviation  from  normal  noticed.  Finally  the  speech  may  be  altered  be- 
cause of  intellectual  deficiencies,  such  as  idiocy,  etc.  On  the  other  hand, 
the  patient's  previous  knowledge  or  illiteracy  should  be  taken  into  account 
in  forming  an  opinion  upon  his  ability  to  write,  read,  etc. 

VI.  REFLEXES 

The  study  of  reflexes  is  of  paramount  diagnostic  importance  in 
neurology. 

A  reflex  action  is  the  result  of  a  peripheral  stimulation.  The  latter 
follows  a  centripetal  course  to  a  center,  from  which  a  motor  act  is  trans- 
mitted through  the  centrifugal  pathway. 

Reflexes  are  divided  into  two  great  groups :  Tendinous  and  cutaneous 
or  deep  and  superficial. 

i.  Normal  Tendon  Reflexes. — To  observe  a  reflex  corrlplete  relaxation 
of  the  muscles  must  be  obtained  before  the  test  is  made. 


72  EXAMINATION   FOR   NERVOUS    DISEASES 

(a)  Patellar  Tendon  Reflex  (Knee-jerk). — The  patient  is  placed  com- 
fortably on  a  chair.  He  is  told  to  relax  his  muscles  and  cross  his  knees. 
When  relaxation  is  difficult  to  obtain,  he  is  engaged  in  a  rapid  conversation 
and  told  not  to  observe  the  test.  The  tendon  is  outlined  and  a  sharp 
blow  is  given  with  the  ulnar  border  of  the  hand  or  with  a  percussion  ham- 
mer. A  contraction  of  the  quadriceps  is  obtained  and  a  forward  jerk  of 
the  leg  follows.  When  the  response  is  very  feeble  or  doubtful,  Jendrassik's 
method  should  be  tried.  The  patient  is  told  to  grasp  firmly  one  hand 
with  the  other  and  pull.  If  the  reflex  is  present,  it  will  then  be  obtained 
or  if  it  is  feeble  with  the  ordinary  test,  will  be  prompt  with  this  methods. 

(b)  Achilles'  Tendon  Reflex. — The  patient  kneels  on  a  chair,  relaxes 
his  muscles  and  a  short  blow  is  given  in  the  preceding  manner  over  the 
tendon.     Normally  there  is  a  plantar  extension  of  the  foot. 

(c)  Triceps  Reflex. — The  patient's  arm  is  held  on  its  anterior  surface 
and  the  forearm  allowed  to  hang  down,  forming  a  right  angle  at  the 
elbow.  The  tendon  of  the  triceps  is  then  percussed.  An  extension 
movement  follows. 

(d)  Biceps  Reflex. — A  semiflexed  and  relaxed  position  is  given  the 
elbow.  A  slight  blow  over  the  tendon  will  be  followed  by  a  flexion 
movement. 

(e)  Masseter  Reflex. — The  mouth  is  slightly  opened,  a  hard  object 
is  placed  on  the  teeth  of  the  lower  jaw.  A  slight  blow  over  the  latter  pro- 
duces a  contraction  of  the  masseter. 

The  above  described  tendon  reflexes  may  be  increased,  diminished  or 
lost.  These  three  conditions  are  extremely  important  for  diagnostic 
purposes.     They  are  indications  of  involvement  of  the  nervous  system. 

Abnormal  Tendon  Reflexes,  (a)  Ankle-clonus.— When  the  patient 
is  seated,  the  calf-muscle  of  his  semiflexed  leg  is  grasped  and  held  gently 
in  the  palm  of  the  hand.  With  the  other  hand  his  foot  is  brought  first 
downward  and  then  abruptly  flexed  dorsally.  A  to  and  fro  movement  of 
the  foot  is  then  produced,  which  may  last  from  a  fraction  of  a  minute 
to  several  minutes. 

(b)  Contra-lateral  Movement. — When  the  patient  lies  on  his  back  with 
the  lower  limbs  semiflexed  and  the  patellar  tendon  is  percussed,  besides 
an  extension  of  the  leg  on  the  same  side,  there  is  also  an  adduction  of 
the  opposite  limb. 

2 .  Normal  Cutaneous  Reflexes.— They  consist  of  muscular  contractions 
produced  by  irritation  of  the  sensory  cutaneous  nerves.  The  contractions 
may  be  limited  to  the  area  stimulated,  but  when  the  stimulation  is  very 
marked  the  contractions  spread  to  other  muscles  and  may  invade  the 
entire  body. 


EXAMINATION   FOR   NERVOUS    DISEASES  73 

(a)  Plantar  Reflex. — When  the  sole  of  the  foot  is  slightly  irritated 
the  toes  flex,  but  when  the  response  is  pronounced  there  will  be  at  first 
a  contraction  of  the  tensor  fascia  lata,  then  a  dorsal  flexion  of  the  entire 
foot,  flexion  of  the  leg  on  the  thigh  and  of  the  thigh  on  the  pelvis. 

(b)  Abdominal  Reflex  (Rosenbach's  Sign). — It  consists  of  a  contrac- 
tion of  the  abdominal  muscles  upon  an  irritation  of  the  skin  of  the  abdomen 
along  the  outer  border  of  the  rectus  abdominalis  muscle. 

(c)  Cremasteric  Reflex.- — It  consists  of  a  sudden  ascension  of  the 
testicle  when  the  skin  of  the  internal  aspect  of  the  thigh  is  stimulated. 

(d)  Anal  Reflex.— Irritation  of  the  perianal  cutaneous  surface  pro- 
duces a  contraction  of  the  sphincter. 

Abnormal  Cutaneous  Reflexes,  (a)  Babinski's  Reflex  (toe  phe- 
nomenon).— It  consists  of  extension  of  the  great  toe  and  sometimes  of 
all  the  toes,  when  the  sole  of  the  foot  is  slightly  irritated.  It  is  best 
elicited  by  stroking  the  outer  margin  of  the  sole  or  across  the  balls  of  the 
toes.  The  extension  of  the  great  toe  may  be  accompanied  by  a  fan-like 
abduction  of  the  other  toes. 

(b)  Oppenheim's  Reflex. — If  the  handle  of  a  percussion  hammer  (or 
any  object)  is  passed  from  above  downward  along  the  inner  border  of 
the  tibia,  producing  at  the  same  time  slight  pressure  upon  the  soft  tissue, 
extension  of  the  great  toe  or  all  the  toes  follows. 

(c)  Paradoxical  Flexor  Reflex  (described  by  the  writer)  consists  of 
extension  of  the  great  toe  or  of  all  the  toes  when  the  deep  muscles  of  the 
calf  are  pressed  upon.  To  elicit  it  the  patient  is  seated  and  his  feet 
(not  the  legs)  are  placed  on  a  stool.  The  limbs  are  everted  and  completely 
relaxed.  The  examiner  places  the  thenar  and  hypothenar  muscles  of  his 
hands  on  the  antero-internal  surface  of  the  tibia  and  turns  his  fingers 
downward  to  the  middle  of  the  calf  muscles.  Pressure  on  the  latter  is 
then  produced.  Instead  of  pressing  the  fingers  may  roll  the  calf  muscles 
from  side  to  side. 

Other  abnormal  reflexes  will  be  described  in  the  respective  chapters 
of  various  organic  diseases. 

Significance  of  the  Abnormal  Tendon  and  Cutaneous  Reflexes. — 
They  are  all  manifestations  of  an  involvement  of  the  motor  area  and  path- 
way. They  are  associated  with  exaggerated  normal  reflexes.  The  para- 
doxical reflex  in  particular,  makes  its  appearance  frequently  long  before 
the  Babinski's  and  Oppenheim's  signs  developed.  It  is  also  present  in 
very  slight  lesions  or  irritation  of  the  motor  tract  and  motor  center. 

Abolition  of  all  reflex  responses  is  observed  in  some  organic  diseases, 
such  as  transverse  lesions  of  the  cord,  in  poliomyelitis,  in  tabes,  finally 
in  coma. 


74  EXAMINATION   FOR   NERVOUS   DISEASES 

VII.  SPHINCTERS 

The  involvement  of  the  sphincters  of  the  bladder  and  rectum  is  a 
frequent  manifestation  in  organic  nervous  diseases.  It  also  occurs  occa- 
sionally in  the  course  of  functional  nervous  disorders. 

Retention,  incontinence,  imperative  and  frequent  micturition  (or 
defecation)  are  the  symptoms  to  be  investigated. 

VIII.  VASOMOTOR  AND  TROPHIC  DISTURBANCES 

They  are  very  frequent  in  nervous  diseases.  Localized  oedema,  local- 
ized adiposis,  local  asphyxia  of  the  extremities,  herpes  zoster,  disturbed, 
function  of  sweat  glands,  bed-sores,  hemiatrophy,  localized  or  generalized 
atrophy — may  all  be  encountered  in  nervous  diseases. 


CHAPTER  III 

CEREBRAL  LOCALIZATIONS 

Anatomically  the  brain  presents  homogeneous  masses  of  gray  and 
white  matter,  but  physiologically  it  is  composed  of  portions,  areas  or 
centers,  the  functions  of  which  are  different  from  each  other.  The  con- 
ception of  centers  was  admitted  even  as  far  back  as  1825.  In  1861 
Broca  first  localized  the  speech  center.  But  a  solid  foundation  to  the  ex- 
istence of  cerebral  localizations  was  laid  by  the  experiments  of  Fritsch 
and  Hitzig  (1870)  and  later  of  Ferrier  on  animals,  and  the  clinical  obser- 
vations of  Hughlings  Jackson.  Since  then  pathological  observations 
began  to  accumulate  and  they  corroborated  the  experimental  researches. 
Thus  regional  diagnosis  of  diseases  of  the  brain  became  facilitated  and 
gained  an  anatomical  basis.  Surgery  benefited  considerably  from  this 
new  acquisition  of  cerebral  physiology. 

MOTOR  CENTERS 

Until  recently  the  motor  area  of  the  cortex  was  considered  as  consist- 
ing of  the  ascending  frontal  and  ascending  parietal  convolutions,  also  the 
paracentral  lobule.  Considerable  doubt  has  arisen  lately  as  to  the  motor 
function  of  the  ascending  parietal  convolution.  First  of  all,  Sherrington 
and  Griinbaum  have  demonstrated  on  anthropoid  apes  that  excitation 
of  the  ascending  parietal  by  strongest  electrical  currents  failed  to  produce 
movements,  while  identical  stimulation  of  the  whole  ascending  frontal 
convolution  and  of  the  Rolandic  fissure  evoked  movements  on  the  opposite 
side  of  the  body.  Also  stimulation  of  the  bases  of  the  frontal  convolu- 
tions was  followed  by  movements  of  the  eyeballs.  Observations  have  also 
heen  made  in  man  as  to  effect  of  electrical  stimulation  of  the  Rolandic  area 
(Mills,  Keen,  Horsley,  Dana,  Cushing,  Lloyd,  Gordon,  etc.).  By  using  the 
unipolar  method  they  obtained  movements  only  from  the  ascending  frontal 
convolutions.  There  are  cases  on  record  showing  that  lesions  of  the  ascend- 
ing frontal  alone  produced  during  life  paralysis  of  the  opposite  side,  while 
no  motor  symptoms  were  present  when  the  lesion  was  found  in  the  post- 
central convolution.  Of  considerable  importance  are  also  the  differences 
in  the  structure  of  postcentral  and  the  precentral  convolutions.  Accord- 
ing to  Campbell  the  cortex  of  the  precentral  convolution  contains  the 

75 


76  CEREBRAL    LOCALIZATIONS 

giant-cells  of  Betz  and  an  abundance  of  nerve-fibers.  The  postcentral 
convolution  has  no  Betz  cells  and  is  not  so  rich  in  nerve-fibers.  The  Betz 
cells  seem  to  be  the  origin  of  the  pyramidal  tracts.  As  a  last  argument 
in  favor  of  the  exclusive  motor  character  of  the  precentral  convolution 
is  found  in  Flechsig's  embryological  studies. 

On  the  other  hand,  there  are  some  facts  which  tend  to  prove  that  the 
so-called  motor  centers  have  also  a  sensory  function,  that  they  are  sen- 
sori-motor.  Horsley  {Brit.  Med.  Jour.,  1909,  p.  125)  removed  the  arm 
center  of  the  precentral  convolution  in  a  patient  suffering  from  convul- 
sions limited  to  the  arm.  The  result  was:  paralysis  of  the  arm,  aster- 
eognosis,  anaesthesia  to  all  forms  of  sensations.  A  year  later  both  motion 
and  general  sensations  improved.  In  view  of  the  absence  of  the  pre- 
central portion  of  the  arm  the  postcentral  controlled  the  movements  and 
sensations.  Clinical  studies  of  motor  paralysis  of  cerebral  origin  show 
that  not  infrequently  sensory  disturbances  are  present  in  the  paralyzed 
limbs.  Tripier,  Golgi,  Exner,  Starr,  Soury,  Dejerine  and  others  have 
given  ample  anatomo-clinical  proofs  of  this  contention.  In  my  personal 
study  {Jour.  Nero,  and  Ment.  Dis.,  1903,  p.  144)  of  35  cases,  I  found  in 
some  a  distinct  parallelism  between  the  motor  and  sensory  disturbances 
in  regard  to  distribution  and  degree.  My  investigations  by  means  of 
faradization  of  the  cortex  {Jour,  of  Amer.  Med.  Assn.,  June,  1907)  show 
that  the  ascending  parietal  convolution  participates  in  formation  of  motor 
area,  although  to  a  considerably  lesser  degree.  In  Rossi  and  Roussy's 
two  cases  {Revue  Neurologique,  1906)  and  in  Souques  and  Barbe's  case 
there  was  a  slight  involvement  of  the  ascending  parietal  convolution. 
Considering  all  the  accumulated  evidences,  clinical,  pathological  and 
experimental,  one  is  more  inclined  to  admit  that  the  motor  and  sensory 
cortical  areas  are  distinct,  that  the  precentral  convolution  is  purely  motor, 
that  the  postcentral  is  only  sensory,  and  that  the  motor  area  includes  the 
precentral,  anterior  part  of  paracentral,  the  bases  of  the  first  two  frontal 
convolutions,  also  the  Rolandic  fissure. 

The  motor  nature  of  the  special  portion  of  the  cortex  is  easily  recog- 
nized from  the  convulsive  and  paralytic  symptoms  produced  by  an  irri- 
tation or  a  destructive  lesion  of  these  centers.  The  latter  control  each 
individual  muscle  or  groups  of  muscles  of  the  opposite  side  of  the  body. 
It  is  therefore  important  to  describe  the  seat  of  each  individual  motor 
center.  For  practical  purposes  it  is  sufficient  to  consider  three  chief 
portions  of  entire  motor  zone  corresponding  to  the  limbs  and  head.  The 
center  for  the  lower  extremities  occupies  the  anterior  part  of  the  para- 
central lobule  and  the  upper  fourth  of  the  ascending  frontal  convolution, 
and  that  of  Rolandic  fissure.    The  two  middle  fourths  represent  the  center 


CEREBRAL    LOCALIZATIONS 


77 


for  the  upper  extremities.  The  center  of  the  head  lies  in  the  lower  fourth 
and  in  the  rolandic  operculum.  The  center  for  the  trunk  lies  between 
those  of  the  extremities. 

Clinical  observations  on  focal  epilepsy  or  electrical  stimulation  of  the 
motor  centers  have  shown  that  in  each  of  the  above  areas  exist  secondary 
centers  which  correspond  to  the  function  of  muscles  of  segments  of  the 
limbs.  Thus  there  are  centers  for  the  shoulder,  elbow,  wrist,  fingers, 
thigh,  knee,  ankle,  toes;  for  the  head  individual  centers  control  the  move- 
ments of  the  face,  tongue,  lips,  pharynx  and  larynx.  The  larynx  has 
two  centers — one  for  each  of  its  two  functions,  viz.  respiration  (abduction 


Fig.  60. — Motor  Centers.     (After  Latnacq.) 


of  the  vocal  cords)  and  phonation  (adduction  of  the  vocal  cords).  The 
center  for  the  conjugate  deviation  of  the  head  and  eyes  lies  in  the  foot  of 
the  second  frontal  convolution.  The  accompanying  illustration  shows 
the  relative  seat  of  these  various  centers. 

It  is  well  understood  that  each  center  controls  muscular  groups  of  the 
opposite  side  of  the  body  and  the  reason  of  it  lies  in  the  decussation  of 
the  pyramidal  fibers.  There  is,  however,  a  certain  group  of  muscles 
which  are  under  the  influence  of  centers  of  both  hemispheres,  in  other 
words,  some  centers  control  simultaneously  muscles  of  both  halves  of 
the  body.  Such  bilateral  centers  exist  for:  (i)  The  muscles  of  the  eye; 
elevation  and  lowering  of  both  eye  globes  are  produced  simultaneously. 
(2)  The    muscles — orbicularis    palpebrarum  and   frontalis.     The  integ- 


78  CEREBRAL    LOCALIZATIONS 

rity  of  the  superior  facial  nerve  in  organic  facial  palsy  (see  hemiplegia) 
is  also  due  to  bilateral  innervation  of  the  upper  part  of  the  face.  (3)  The 
muscles  of  mastication.  (4)  Some  muscles  of  the  tongue.  (5)  Muscles 
of  deglutition.  (6)  Muscles  of  the  larynx.  (7)  Muscles  of  respiration — 
diaphragm  and  intercostal. 

SPEECH  CENTERS 

They  are  situated  along  the  Sylvian  fissure  and  in  the  left  hemisphere 
for  right-handed,  in  the  right  hemisphere  for  left-handed  individuals. 
There  are  four  speech  centers:  one  serves  to  receive  words,  another  to 
receive  read  words,  a  third  to  express  in  an  articulate  language  what  is 
heard  or  seen.  Expressing  what  is  heard  or  seen  can  also  be  done  by 
writing.  Hence  a  fourth  center  has  been  added  to  the  above  three. 
These  four  centers  are  associated  with  each  other  by  commissural  fibers 
of  great  importance,  so  that  destruction  of  one  leads  to  interruption  of 
its  communication  with  others.  In  the  chapter  on  aphasia  various  phe- 
nomena of  speech  produced  by  the  destruction  of  these  anatomical  con- 
nections will  be  discussed  at  length. 

The  localization  of  the  four  speech  centers  is  distributed  as  follows: 

The  center  for  auditory  images,  viz.  for  comprehending  spoken  words, 
occupies  the  posterior  fourth  of  the  first  temporal  convolution  (left). 
A  lesion  of  this  center  causes  a  form  of  sensory  aphasia  known  as  "word 
deafness. " 

The  center  for  visual  graphic  images,  viz.  for  distinguishing  written 
or  printed  words,  occupies  the  left  angular  gyrus  (postero-inferior  part 
of  the  left  parietal  lobe).  A  lesion  of  this  center  produces  a  form  of 
sensory  aphasia  known  as  "word  blindness"  (alexia).  These  two 
centers,  a  lesion  of  which  is  responsible  for  "sensory  aphasia,"  are  known 
as  Wernicke's  area  and  the  speech  defect  as  Wernicke's  aphasia. 

The  center  for  the  faculty  of  writing  lies  in  the  foot  of  the  second 
frontal  convolution,  a  lesion  of  which  produces  inability  to  write — 
agraphia. 

The  motor  center  for  pronouncing  and  articulating  words,  as  discov- 
ered by  Broca,  occupies  the  foot  of  the  third  frontal  convolution  (left), 
immediately  in  front  of  the  centers  of  phonation  utilized  in  speech.  The 
destruction  of  this  center  produces  motor  aphasia  (aphemia).  The 
speech  and  writing  functions  are  frequently  involved  together  because  of 
the  proximity  of  their  two  centers  and  of  the  commissural  fibers  connect- 
ing these  centers  with  the  auditory  and  visual  centers.  As  to  the  new 
view  advanced  recently  by  P.  Marie,  see  chapter  on  Aphasia. 


CEREBRAL    LOCALIZATIONS  79 


SENSORY  CENTERS 


(a)  General  Sensations  (touch,  pain,  temperature,  muscular  sense). — 
Although  the  question  is  still  somewhat  debatable,  it  is  nevertheless  ad- 
mitted by  the  majority  of  competent  writers  that  the  motor  and  sensory 
spheres  of  the  cortex  are  separate.  In  the  chapter  on  motor  center  it 
was  shown  that  the  prevalent  opinion  is  presently  in  favor  of  the  ascending 
parietal  convolution  being  the  center  for  general  sensations.  The  parie- 
tal lobe  presents  three  important  portions:  superior  lobule,  supramar- 
ginal  lobule  and  angular  gyrus.  The  parietal  lobe  forms  a  part  of  Flech- 
sig's  posterior  association  area.  The  superior  parietal  lobule  together  with 
the  postcentral  convolution  can  be  considered  as  a  cortical  center  for  gen- 
eral sensibility.  An  irritative  lesion  in  this  area  will  cause  Jacksonian 
epilepsy  with  a  sensory  aura.  Flechsig  believes  that  the  sensory  cortical 
area  corresponds  to  the  large  surface  sensory  area  which  comprises  the 
skin,  mucous  membrane,  muscles,  articulations  and  viscera.  All  forms 
of  sensations  (touch,  pain,  temperature  and  muscular  sense)  are  repre- 
sented in  this  center,  but  nothing  is  known  in  regard  to  a  separate  local- 
ization for  each  individual  form.  There  is  reason  to  believe  that  the  sen- 
sory area  contains  also  centers  for  the  nerves  of  secretory  glands  and  for 
vaso-motor  nerves  (Adamkiewicz,  Bechterew). 

Stereognostic  Sense. — Under  this  term  is  understood  the  faculty  of 
recognizing  the  form,  size  and  consistency  of  objects  by  touch.  To  elicit 
it  various  objects  are  placed  separately  in  each  hand  of  the  patient,  his 
eyes  being  closed.  Inability  to  recognize  the  characteristics  of  the  ob- 
jects constitutes  asterognosis.  Although  muscular  sense,  tactile  and  tem- 
perature sensations,  also  those  of  weight  and  pressure,  participate  in 
perception  of  an  object  placed  in  the  hand,  stereognosis  is  nevertheless 
considered  as  a  special  sense  which  has  a  special  cortical  center.  It 
was  mentioned  on  page  69  that  in  spite  of  the  integrity  of  various 
elementary  sensibilities  the  stereognostic  sense  may  be  abolished.  As- 
tereognosis  has  been  often  observed  in  lesions  of  the  superior  parietal 
lobule  (Mills,  Burr,  Starr  and  others)  or  of  the  supramarginal  gyrus. 
This  is,  however,  not  the  exclusive  seat  of  the  stereognostic  sense,  as  in 
lesions  of  the  Rolandic  area  asterognosis  has  also  been  noted.  On  the 
other  hand,  in  lesions  of  the  parietal  lobe  the  stereognostic  sense  has  also 
been  found  intact  as  it  can  be  seen  from  my  case  {Med.  Record,  1908) 
in  which  a  bullet  entered  the  left  parietal  region  and  subsequent  operative 
procedures  lacerated  very  extensively  the  superior  parietal  lobule  without 
involving  this  special  sense. 


80  CEREBRAL    LOCALIZATIONS 


SPECIAL  SENSATIONS 


(a)  Visual  Centers. — The  cortical  centers  for  vision  occupies  the  cu- 
neus  and  the  calcarine  fissure  in  either  hemisphere.  Most  frequently  a 
lesion  of  this  area  in  one  hemisphere  will  produce  a  hemianopsia,  viz. 
a  blindness  in  one-half  of  both  eyes  on  the  side  opposite  to  the  lesion 
and  total  blindness  if  the  lesion  is  bilateral.  The  convexity  of  the  occipi- 
tal lobe  is  also  concerned  in  vision  so  that  hemianopsia  may  also  be  the 
result  of  a  lesion  of  the  occipital  cortex. 

The  occipital  lobe  is  connected  with  sub-cortical  centers  of  the  visual 
apparatus,  viz.  with  anterior  quadrigeminal  bodies,  pulvinar  and  external 
geniculate  body — by  means  of  the  fibers  of  optic  radiations,  which  pass 
through  the  retro-lenticular  segment  of  the  internal  capsule  (see  An- 
atomy). Does  a  lesion  of  any  of  these  portions  cause  blindness  of 
the  visual  field?  The  most  recent  investigations  show  that  an  involve- 
ment of  the  optic  radiations,  of  the  chiasma  or  of  the  optic  nerve  produces 
hemianopsia,  a  lesion  of  the  pulvinar  may  (exceptionally)  do  it,  but  there 
are  no  indisputable  proofs  for  hemianopsia  due  to  diseases  of  the  quadri- 
geminal bodies  or  of  external  geniculate  body.  As  to  the  angular  gyrus, 
it  is  the  center  for  word-seeing.  A  lesion  of  it  will  produce  word-blind- 
ness (alexia)  (see  chapter  on  Aphasia) . 

(b)  Auditory  Centers. — Very  few  cases  of  pure  deafness  came  to  au- 
topsy, but  they  show  that  the  cortical  center  of  hearing  is  in  the 
temporal  lobe.  According  to  Ferrier,  Seppilli  and  others,  the  first  tem- 
poral convolution  is  particularly  concerned  in  the  function  of  hearing. 
Flechsig,  Bechterew  and  Monakow  have  shown  that  the  posterior  quad- 
rigeminal bodies,  the  internal  geniculate  body  are  in  relation  with  the 
cortex  of  the  temporal  lobe,  consequently  with  the  auditory  area.  The 
function  of  "word-hearing"  (see  above)  is  not  necessarily  connected  with 
that  of  hearing  in  general,  as  patients  affected  with  "word  deafness"  are 
not  deaf,  generally  speaking.  It  is,  therefore,  wise  perhaps  to  conclude 
that  while  "word  deafness"  is  associated  with  a  lesion  only  of  the  left 
hemisphere,  the  general  audition  is  controlled  by  bilateral  centers. 
Broadbent  observed  a  case  of  a  deaf-mute  in  whom  atrophy  of  both 
superior  temporal  gyri  was  found.  Mills  describes  a  case  of  a  woman 
deaf  for  thirty  years  in  whom  both  superior  temporal  gyri  were 
atrophied. 

(c)  Olfactory  and  Gustatory  Centers. — Long  ago  Ferrier  observed 
from  his  experiments  that  destruction  of  the  apices  of  both  temporal 
lobes  led  to  loss  of  sense  of  taste  and  smell  (anosmia).  Some  writers 
corroborated    this    observation.     According    to    Flechsig    the    olfactory 


CEREBRAL    LOCALIZATIONS  8 1 

center  is  in  the  cornu  Airimonis  and  hippocampus.  Campbell's  histolog- 
ical investigations  (Cambridge  University  Press,  1905)  suggest  that  the 
lobus  pyriformus,  which  consists  of  the  external  root  of  the  olfactory 
lobe  and  of  the  gyrus  hippocampi,  must  be  considered  as  the  principal 
cortical  center  of  the  olfactory  sense.  On  the  other  hand,  some  anatomo- 
clinical  observations  point  to  the  uncinate  gyrus  as  the  cortical  seat  of 
taste  or  smell  senses.  J.  Hughlings  Jackson  speaks  of  cases  of  Epilepsy 
with  intellectual  aurae  (dreamy  state)  associated  with  sensations  of  smell. 
Buzzard  in  1905  reports  similar  cases  in  which  lesions  were  found  in  tem- 
poro-sphenoidal  lobes  {Lancet,  1906,  p.  1808).  Although  the  few  records 
found  in  the  literature  speak  in  favor  of  the  mentioned  cortical  areas,  nev- 
ertheless there  is  no  absolute  proof  of  the  existence  of  cortical  centers  for 
these  two  special  senses.  Zuckerkandl's  observation  is,  however,  signifi- 
cant in  this  respect.  Animals  with  a  highly  developed  sense  of  smell  pos- 
sess a  large  limbic  lobe  (see  Anatomy) ;  the  latter  is  atrophied  in  animals 
whose  olfactory  sense  is  rudimentary  or  absent. 

Center  for  Intelligence. — There  is  no  unanimous  opinion  as  to  the  local- 
ization of  human  thought  in  the  brain.  Intelligence  is  the  result  of  func- 
tion of  all  the  centers  combined  and  of  the  association  fibers  which  serve  to 
connect  the  centers.  However,  observations  made  by  Flechsig,  Hitzig, 
Ferrier  and  others  point  to  the  prefrontal  lobe  as  the  most  prominent  part 
of  the  brain  where  superior  psychic  processes  are  elaborated.  The 
history  of  tumors  or  of  other  lesions  of  the  prefrontal  area  seem  to  cor- 
roborate this  view  (see  Tumors  of  Brain,  also  my  article  on  Function  of 
Prefrontal  Lobes  in  Jour.  Amer.  Med.  Ass.,  1907).  Mabille  and  Pitres 
(Revue  de  Med.,  No.  4,  1913)  report  the  history  of  a  patient  who  lost  the 
ability  of  recalling  perfectly  events  of  his  past  life,  of  fixing  any  new 
memories,  also  of  appreciating  his  present  surroundings.  Autopsy 
showed  two  symmetrical  areas  of  softening,  one  in  each  hemisphere  in 
the  midst  of  the  white  substance  of  the  prefontal  lobes; 


CHAPTER  IV 

APOPLEXY 

HEMORHAGE.    EMBOLISM.    THROMBOSIS 

This  morbid  condition  is  characterized  usually  by  a  sudden  loss  of 
consciousness  with  complete  or  partial  loss  of  power  and  sensations  on 
one  side  of  the  body  (in  some  cases  consciousness  is  preserved). 

Morgagni  was  the  first  to  show  that  apoplexy  has  an  anatomical  reason 
in  the  brain  or  on  its  surface.  For  a  long  time  apoplexy  and  cerebral 
hemorrhage  were  considered  synonymous,  but  in  1819  a  new  era  in  the 
history  of  the  subject  commenced  with  a  series  of  anatomopathological 
works  which  rapidly  followed  one  another,  all  tending  to  prove  that  other 
factors  besides  hemorrhages  are  apt  to  cause  apoplexy.  The  most  com- 
mon immediate  causes  as  accepted  at  the  present  are:  Hemorrhage, 
Embolism  and  Thrombosis. 

These  three  conditions  lead  to  one  final  result,  namely  hemiplegia, 
but  the  onset,  the  course  of  the  disease  and  the  pathological  lesions  are 
not  identical.     A  separate  description  is  therefore  justifiable. 

A.    HEMORRHAGE 

Pathology. — Rupture  of  a  blood  vessel  may  occur  in  the  brain  sub- 
stance or  on  its  surface.  The  most  frequent  seat,  however,  is  the  internal 
capsule  and  the  neighboring  central  ganglia  between  which  the  capsule 
is  located  (see  Anatomy,  page  31).  The  reason  of  it  lies  in  the  fact  that 
the  small  vertical  arteries  which  are  distributed  to  these  parts  (namely 
the  lenticulo-striate  and  lenticulo-thalamic  arteries),  having  no  collateral 
branches,  are  under  a  relatively  high  pressure  and  therefore  are  more  apt 
to  rupture  than  the  cortical  vessels,  for  example,  which  branch  a  great 
deal.  Durand-Fardel  has  shown  that  these  same  arteries  develop  fre- 
quently miliary  aneurisms,  which  means  degeneration  of  vessel  wall  and 
hence  rupture.  They  were  deservedly  called  by  Charcot  "arteries  of 
cerebral  hemorrhage."  Degenerative  conditions  of  the  blood  vessels 
are  therefore  the  chief  factor  in  cerebral  hemorrhage.  When  the  vessel- 
wall  is  altered  (peri-endarteritis),  miliary  aneurisms  are  apt  to  form 
(Charcot  and  Bouchard).  But  the  important  exciting  element  is  the 
rise  of  arterial  pressure,  which,  when  it  reaches  the  short  terminal  arteries 
of  the  central  ganglia,   facilitates   their  rupture.     In  aged  individuals 

82 


APOPLEXY 


83 


Ferrand  called  attention  to  a  special  state  of  cerebral  tissue  characterized 
by  small  cavities  (lacunes)  in  the  center  of  which  is  seen  an  artery.  The 
cavities  are  due  to  a  disintegration  of  nervous  elements.  As  the  blood 
pressure  is  invariably  increased  in  advanced  age,  the  atheromatous  cen- 
tral artery  is  easily  ruptured  when  the  blood  vessel  is  under  high  tension. 


Fig.  61. — Extensive  Hemorrhage  in  the  Anterior  Cornu  oe  the  Left  Lateral 
Ventricle.     Sudden  Death.     (Original.) 

The  clot  which  forms  immediately  after  a  cerebral  hemorrhage  and 
the  adjacent  brain  tissue  undergoes  certain  changes.  The  red  color  of 
the  clot  gradually  changes  into  yellow.  The  nervous  tissue,  which  is 
affected  by  the  clot,  is  liquefied  and  eventually  becomes  absorbed;  the 
neighboring  tissue,  being  compressed,  suffers  in  its  turn;  the  connective 
tissue  and  the  neuroglia  proliferate  and  form  a  thick  capsule  around  the 


84 


APOPLEXY 


softened  area  and  a  cyst  containing  a  yellowish  fluid  is  the  result.  When 
the  contents  of  the  cyst  is  absorbed  its  walls  shrink  and  a  cicatrix  is  formed. 
Frequently  the  course  of  events  is  not  so  favorable.  The  ruptured  artery 
continues  to  ooze  and  the  destruction  of  brain  tissue  continues.     If  the 


Fig.  62. — Extensive  Hemorrhage  in  Both  Hemispheres.     Rapid  Death.     (Original.) 

hemorrhage  is  not  stopped,  it  may  reach  the  ventricles:  various  struc- 
tures of  the  brain  undergo  then  considerable  pressure  (Figs.  61  and  62). 

When  a  hemorrhage  occurs  on  the  surface  of  the  brain,  beneath  or 
within  the  membranes,  the  clot  may  not  become  organized  and  destruc- 
tion of  cortical  tissue  may  be  avoided,  so  that  the  brain  will  resume  its 
function  when  the  clot  is  removed. 


APOPLEXY  85 

The  most  important  consequence  of  cerebral  hemorrhage  is  its  remote 
effect  on  the  nervous  tissue  beneath  and  in  the  immediate  vicinity  of 
the  area  destroyed  by  the  clot.  Secondary  degenerations,  which  promptly 
set  in,  are  the  usual  result.  They  may  be  traced  through  an  entire  tract. 
A  hemorrhage,  for  example,  in  the  posterior  limb  of  the  internal  capsule 
will  be  followed  by  a  descending  degeneration  in  the  entire  motor  path- 
way, even  in  the  lowest  portion  of  the  cord.  The  degeneration  is 
characterized  at  first  by  disappearance  of  the  myelin  and  nerve-fibers 
which  are  ultimately  replaced  by  connective  tissue,  and  a  scar  is  thus, 
formed. 

Etiology. — Disease  of  the  blood  vessels  is  the  chief  cause  of  hemorrhage. 
The  most  important  factors  producing  changes  in  the  vessel  walls  are: 
advanced  age,  intoxications  and  infection.  Although  no  age  is  exempt 
from  cerebral  hemorrhage,  nevertheless  degenerative  condition  of  arteries, 
resulting  in  arteriosclerosis  is  proper  to  the  degenerative  period  of  life 
and  the  majority  of  cases  of  cerebral  hemorrhages  occur  after  forty  years 
of  age.  According  to  the  records  of  St.  Bartholomew's  Hospital  the 
largest  percentage  is  between  the  ages  of  forty  and  fifty  and  decrease 
with  the  following  decades.  Among  intoxications,  syphilis  and  lead  occupy 
the  first  place  and  their  influence  is  augmented  when  they  are  present  in 
advanced  life.  Alcohol  also  predisposes  to  atheromatous  state  of  blood 
vessels.  Degenerative  conditions  of  cerebral  blood  vessels  are  also  ob- 
served in  pernicious  anemia,  purpura,  scurvy;  in  Bright's  disease;  finally 
in  conditions  producing  cardiac  hypertrophy,  which  cannot  be  com- 
pensated. 

As  to  sex,  men  are  more  liable  to  cerebral  hemorrhage  than  women. 
Sometimes  a  hereditary  predisposition  is  observed.  In  a  case  under  my 
observation,  a  young  man  of  twenty-seven  died  suddenly  after  a  violent 
paroxysm  of  whooping-cough  from  ventricular  hemorrhage.  His  arteries 
and  other  organs  were  found  totally  normal.  Three  members  of  his 
family  died  from  apoplexy  when  young  and  in  good  health  (Fig.  62). 

In  the  majority  of  cases  of  cerebral  hemorrhage  the  altered  vessel  wall 
will  rupture  under  the  influence  of  raised  blood  pressure  and  this  occurs 
in  severe  muscular  efforts,  as,  for  example,  in  lifting  heavy  weights,  in 
the  act  of  difficult  defecation,  in  parturition,  in  paroxysms  of  severe  cough, 
coitus,  attacks  of  anger,  excitement,  etc.  Finally,  intra-cerebral  hemor- 
rhage may  occur  irrespective  of  arterial  alterations,  namely  from  injuries 
to  the  head:  fractures  of  the  skull,  blows  or  a  simple  contusion.  Hemor- 
rhages on  the  surface  of  the  brain  are  usually  the  result  of  direct  cranial, 
trauma,  although  it  may  occur  under  the  conditions  related  above. 


86 


APOPLEXY 


B.  SOFTENING  OF  THE  BRAIN   (EMBOLISM  AND  THROMBOSIS) 

Pathology. — Cerebral  softening  is  the  result  of  an  interruption  of  blood 
supply  which  is  usually  produced  by  an  embolus  or  thrombus.  When  this 
occurs,  the  first  effect  will  be  a  local  anemia  and  a  change  in  the  consistency 
of  the  tissue  supplied  by  the  obstructed  vessel.     At  the  end  of  twenty-four 


Fig.  63. — Extensive  Softening  in  the  Left  Hemisphere,  Involvtvg  the  Internal 
Capsule,  Basal  Ganglia  and  Corona  Radiata.     (Original.) 

hours  effusion  of  serum  (oedema),  breaking  up  of  nerve  elements  and 
softening  take  place.  The  color  of  the  affected  area  is  at  first  white  be- 
cause of  the  local  anemia,  but  later  on,  when  the  return  circulation  is 
established,  some  corpuscles  escape  with  the  serum  under  high  pressure 


APOPLEXY  87 

into  the  tissue  and  give  the  softened  area  a  red  color  (red  softening) 
which  still  later  (in  the  fourth  week)  changes  to  yellow  because  of  the  de- 
generative changes  in  the  effused  blood.  Yellow  and  red  softening  is 
observed  chiefly  in  the  convolutions,  viz.  in  the  gray  matter;  white 
softening  in  the  white  substance  of  the  brain. 

In  but  a  few  days  after  an  onset  the  brain  tissue  is  in  a  state  of  de- 
generation. Cells,  neuroglia,  myelin  are  dissociated,  and  the  leucocytes 
which  escape  from  the  capillaries  are  in  abundance.  The  entire  affected 
area  undergoes  fatty  degeneration.  In  an  advanced  state  of  degeneration 
(red  softening)  punctiform  hemorrhages  in  the  midst  of  softened  tissue 
are  seen.  Yellow  softening  belongs  to  old  cases:  the  coloring  matter  of 
the  blood  is  modified  and  the  softened  area  is  transformed  into  fatty  sub- 
stance. The  ultimate  result  of  softening  is  either  formation  of  a  cyst  in 
the  place  of  the  softened  focus  or  formation  of  a  cicatrix.  The  latter  is 
due  to  proliferation  partly  of  the  neuroglia  and  partly  of  the  adventitia 
of  the  blood  vessels.  As  to  the  obstruction  in  the  vessel  itself,  it  is  con- 
stituted either  by  a  clot  or  a  particle  of  fibrin  carried  from  the  diseased 
valves  of  the  heart,  or  by  a  fragment  of  an  atheromatous  plaque.  The  clot 
usually  undergoes  changes,  degenerates  and  sometimes  calcifies.  The 
obstructed  blood  vessel  may  become  a  fibrous  cord.  As  some  cases  of 
embolism  recover,  it  is  probable  that  the  collateral  circulation  interferes 
in  such  cases  and  assists  in  keeping  up  the  nutrition  of  the  area  supplied 
by  the  blocked  artery.  In  other  cases  sudden  death  may  occur  long 
after  the  onset  of  the  apoplexy.  Aneurisms  were  found  in  these  cases  on 
one  side  of  the  embolus  and  death  was  due  to  their  rupture.  In  throm- 
bosis recovery  cannot  take  place,  because  the  diseased  vessel  is  surrounded 
by  other  diseased  vessels  (atheroma)  and  collateral  circulation  is  of  no 
special  usefulness. 

Softening  may  occur  in  any  part  of  the  brain,  but  the  most  frequent 
seat  is  in  the  basal  ganglia  and  internal  Capsule.  The  left  hemisphere 
is  more  frequently  affected  by  embolism  than  the  right.  The  middle 
cerebral  arteries  and  their  branches  are  the  usual  seat  for  embolism  and 
the  arteries  at  the  base  for  thrombosis,  (Fig.  63). 

Etiology — Embolism  and  thrombosis,  which  are  the  causes  of  cerebral 
softening,  have  a  different  etiology.  In  a  general  way  it  can  be  said  that 
embolism  is  of  cardiac  origin,  while  thrombosis  is  of  arterial  origin.  In 
the  majority  of  cases  an  inflammatory  condition  of  the  heart  (endocarditis) 
is  the  cause  of  embolism,  although  an  embolus  may  come  from  other 
sources,  from  the  lungs,  for  example.  Vegetations  on  the  mitral  valve, 
clots  from  a  diseased  heart  may  become  detached  in  a  physical  effort,  in  a 
shock  or  a  severe  cough  and  thrown  into  circulation,  producing  an  embol- 


88  APOPLEXY 

ism  in  the  brain.  In  ulcerative  endocarditis  the  detached  particles  will 
carry  microorganisms  into  the  cerebral  arteries  and  thus  produce  besides 
embolism  an  inflammation  of  the  surrounding  tissue.  A  disease  of  the 
pulmonary  vein,  a  disease  of  the  lung,  such  as  empyema,  are  apt  to  send 
embolic  clots  into  the  cerebral  blood  vessels. 

In  thrombosis  the  primary  lesion  lies  in  the  arteries.  An  endarteritis 
narrows  or  obliterates  the  lumen  of  the  blood  vessel,  coagulation  of  the 
blood  follows.  The  usual  causes  of  formation  of  thrombus  are  syphilis 
in  young  individuals,  arteriosclerosis  in  advanced  age,  chronic  intoxication, 
such  as  alcohol,  lead,  etc.  There  is  another  condition  which  deserves 
special  mention  that  leads  to  thrombosis,  viz.  weakness  of  the  myocardium 
with  the  resulting  retardation  of  circulation.  This  is  seen  in  cachexia 
without  atheromatous  changes  in  the  blood  vessels,  in  infectious  diseases, 
in  chlorosis,  in  typhoid  fever,  in  diphtheria,  in  pneumonia.  The  state 
of  blood  in  diabetes  and  gout  favors  its  coagulation  and  therefore  thrombo- 
sis. In  old  individuals  unusual  exertion  is  apt  to  produce  thrombosis; 
the  latter  commonly  occurs  in  the  night  during  sleep  after  fatigue. 

The  question  as  to  the  cause  of  the  development  of  thrombosis  in  these 
diseases  is  not  entirely  settled.  It  is,  however,  probable  that  micro- 
organisms when  present  in  the  blood  increase  its  coagulability  or  develop 
an  endarteritis,  which  leads  to  formation  of  thrombi. 

Symptoms  (of  Hemorrhage,  Embolism  and  Thrombosis). — The 
most  conspicuous  manifestation  of  apoplexy  among  other  symptoms 
is  a  paralysis  of  one  side  of  the  body.  The  onset,  the  course  and  the 
accompanying  symptoms  may  vary  in  apoplectic  seizures  caused  by 
embolism,  thrombosis  and  hemorrhage,  but  the  inevitable  result  of  all 
these  conditions  is  almost  always  hemiplegia.  It  is  therefore  appropriate 
to  indicate  first  the  differences  and  then  conclude  with  a  chapter  on 
hemiplegia. 

Symptoms  of  Hemorrhage 

An  apoplectic  stroke  may  be  preceded  by  prodromes,  may  set  in  sud- 
denly or  gradually;  with  or  without  loss  of  consciousness.  The  usual  pre- 
cursory symptoms  are:  vertigo,  sensation  of  fullness  in  the  head,  headache, 
tingling  in  one-half  of  the  body,  nausea,  vomiting,  ringing  in  the  ears  and 
speech  disturbance.  The  loss  of  consciousness  follows  rapidly.  The 
latter  may  present  various  degrees;  in  some  case  it  is  absolute,  in  others 
partial.  The  comatose  state  in  which  the  patient  is  found  after  a  stroke 
is  usually  attended  by  a  loss  of  all  voluntary  movements  and  sensations, 
by  a  congestion  of  the  face,  by  a  difficult,  superficial  and  noisy  breathing. 
The  pupils  are  usually  dilated  and  do  not  react  to  light. 


APOPLEXY  89 

In  view  of  the  hemiplegia  which  ordinarily  follows  apoplexy  the  cheek 
on  the  paralyzed  side  is  flaccid  and  puffed  out  during  each  act  of  expiration. 
The  cutaneous  reflexes  are  abolished.  Frequently  there  is  involuntary 
evacuation  of  urine  and  feces.  The  temperature  drops  down  one  or 
two  degrees  at  the  beginning,  but  a  few  hours  later  rises  to  normal;  in 
fatal  cases  it  may  rise  to  1050  and  even  to  1080.  In  fact,  elevation  of 
temperature  in  the  course  of  cerebral  apoplexy  is  of  a  very  grave  omen. 
If  recovery  is  to  follow,  the  patient  recovers  consciousness;  symptoms  of 
amelioration  begin  to  appear  at  the  end  of  *  twenty-four  or  forty-eight 
hours.  Pulse,  respiration,  temperature  and  the  various  disturbed  func- 
tions gradually  return  to  normal.  Then  symptoms  of  local  paralysis 
become  evident.  They  will  be  discussed  in  detail  in  the  chapter  on 
hemiplegia.  In  grave  cases  the  loss  of  consciousness  may  become  deeper 
and  deeper  and  the  coma  may  last  for  days  before  death  (Ingravescent 
apoplexy).  Irregular  breathing  during  the  coma  and  especially  of 
Cheyne-Stokes'  type  presents  a  grave  outlook.  The  same  can  be  said 
of  an  irregular  pulse. 

There  is  an  important  symptom  which  deserves  special  mention,  as 
it  frequently  accompanies  apoplexy  and  has,  in  my  opinion,  a  diagnostic 
value.  The  symptom  is  called  "conjugate  deviation  of  the  head  and 
eyes."  At  the  onset,  especially  of  cerebral  hemorrhage,  the  deviation  of 
the  head  and  eyes  is  toward  the  paralyzed  side.  When  the  irritating 
lesion  is  only  temporary,  the  condition  will  gradually  change:  the  patient 
will  turn  head  and  eyes  toward  the  lesion;  the  latter  is  the  most  com- 
mon attitude.  Milian  (Pr  ogres  Medical,  1908,  No.  18)  has  recently  called 
attention  to  two  symptoms  which  during  the  comatose  state  will  enable 
to  determine  the  side  which  is  going  to  be  paralyzed.  They  are:  loss  of 
the  abdominal  reflex  and  of  the  corneal  reflex. 

Symptoms  of  Embolism  and  Thrombosis 

The  apoplectic  stroke  is  not  as  intense  and  not  as  durable  as  in  cere- 
bral hemorrhage.  The  loss  of  consciousness,  which  is  constant  in  hemor- 
rhage, is  frequently  missing  here.  If  the  lumen  of  a  large  artery  is  oc- 
cluded, sudden  loss  of  consciousness  will  follow,  but  in  occlusions  of  small 
arteries  with  emboli  and  in  thrombosis  of  arteries  of  any  size  no  loss  of 
consciousness  will  occur. 

Whether  the  loss  of  consciousness  is  present  or  .absent,  the  onset  in 
both  cases  may  be  sudden  or  gradual,  although,  speaking  generally,  in 
embolism  a  gradual  onset  is  rare.  The  symptoms  of  coma,  when  the 
latter  is  present,  are  milder  and  sometimes  opposite  to  those  of  coma  in 


QO  APOPLEXY 

hemorrhage  (the  pulse  is  not  retarded,  face  is  pale,  temperature  rises 
immediately).  When  the  onset  is  gradual  prodromal  symptoms  are 
always  present.  Twenty-four  or  forty-eight  hours  prior  to  the  onset  of 
the  paralysis  there  is  a  sensation  of  numbness  or  slight  pain  in  the  ex- 
tremity or  extremities  which  are  to  be  paralyzed.  This  is  soon  followed 
by  a  weakness  at  first  in  the  fingers  or  toes,  which  gradually  progresses 
and  involves  the  entire  limb.  The  paretic  condition  becomes  a  genuine 
paralysis  at  the  end  of  two  days.  Sometimes  collateral  circulation  in- 
tervenes and  then  amelioration  of  symptoms  is  seen  instead  of  complete 
paralysis.  It  is  therefore  advisable  to  reserve  the  prognosis  for  two  or 
three  days.  The  gradual  onset  of  apoplexy,  due  to  a  gradual  softening, 
is  of  course  met  with  in  the  progressive  arterial  degeneration  of  advanced 
age. 

The  moderate  intensity  and  durability  are  not  the  only  characteristic 
features  of  apoplectic  seizures  caused  by  cerebral  softening.  When  the 
obstructed  blood  vessel  is  cortical,  instead  of  coma  epileptiform  con- 
vulsions set  in;  they  may  be  focal  and  limited  to  the  limb  or  limbs,  which 
will  eventually  become  paralyzed,  or  else  generalized. 

Hemiplegia 

As  mentioned  above,  paralysis  is  the  final  result  of  apoplectic  seizures 
in  hemorrhage,  embolism  and  thrombosis.  When  the  paralysis  affects 
one-half  of  the  body,  the  condition  is  hemiplegia.  The  latter  is  total 
when  the  face  is  involved  in  addition  to  the  arm  and  leg.  The  loss  of 
power  may  be  complete  or  incomplete.  When  one  limb  is  affected, 
the  condition  is  called  monoplegia. 

Two  phases  should  be  considered  in  the  course  of  hemiplegia.  One  is  that 
of  flaccidity,  the  other  that  of  rigidity  with  later  contractures.  At  the  onset 
and  a  short  time  after  the  onset  flaccidity  is  the  main  feature.  When 
the  arm  and  leg  are  raised  and  abandoned,  they  fall  as  inert  bodies;  all 
movements  are  abolished.  Gradually,  however,  the  power  returns  and 
this  is  noticed  earlier  in  the  leg  than  in  the  arm,  so  that  in  a  well-established 
hemiplegia  the  arm  is  usually  more  involved  than  the  leg.  As  to  the 
individual  muscles,  the  extensors  are  usually  more  affected  than  the 
flexors  and  the  abductors  more  than  the  adductors.  The  deep  reflexes, 
which  at  the  beginning  were  abolished,  soon  return  and  become  increased: 
the  knee-jerks,  the  tendo  Achillis'  reflex,  the  biceps  and  triceps  reflexes 
also  the  wrist  reflex  of  the  upper  extremities  and  the  jaw-reflex  are  all  very 
active. 

The  presence  of  ankle-clonus  during  the  flaccid  phase  of  the  paralysis 


APOPLEXY  91 

is  not  constant.  [This  phenomenon  when  present  is  elicited  in  the 
following  manner:  the  patient's  leg  is  placed  in  a  semi-flexed  position  on 
the  palmar  surface  of  the  left  hand  of  the  observer;  the  latter  grasps  with 
his  right  hand  the  sole  of  the  foot,  extends  it  and  abruptly  flexes  it  on  the 
ankle;  rapid  clonic  movements  of  the  foot  follow.]  Babinski  described  a 
toe  phenomenon  which  may  make  its  appearance  even  immediately  after 
the  onset  of  hemiplegia.  It  consists  of  extension  of  the  great  toe  or  of  all 
the  toes  when  the  sole  of  the  foot  is  gently  irritated.  This  sign  is  of  great 
diagnostic  value,  as,  according  to  the  generally  accepted  view,  it  is  present 
whenever  the  motor  area  or  the  motor  pathway  in  the  brain  or  cord  are 
diseased  or  only  disturbed  in  their  function.  There  is  another  sign 
which  was  first  described  by  the  writer  and  which  is  also  frequently 
observed  at  the  onset  of  hemiplegia.  It  consists  of  extension  of  the  great 
toe  or  of  all  the  toes  when  the  calf  muscles  of  the  leg  are  deeply  pressed 
upon;  by  this  procedure  the  flexor  muscle-group  is  brought  into  action, 
and  since  the  result  is  extension  instead  of  flexion  I  gave  it  the  name 
"paradoxical  flexor  reflex."  The  latter  is  found  in  the  same  conditions 
as  Babinski's  sign.  Moreover,  it  was  found  in  a  large  number  of  cases  of 
hemiplegia  before  Babinski's  sign  made  its  appearance.  (See  also  page 
73.)  Oppenheim's  sign  which  consists  of  extension  of  the  great  toe  when 
downward  pressure  is  produced  over  the  extensors  of  the  leg  close  to  the 
tibia,  is  also  sometimes  present  in  hemiplegia.  Mendel  observed  that 
percussion  of  the  external  aspect  of  the  dorsum  of  a  hemiplegic  foot, 
there  is  a  flexion  of  the  four  last  toes,  but  especially  of  the  second  and 
third  toes.     In  normal  condition  there  is  extension. 

Babinski's  toe  phenomenon  may  be  also  obtained  by  striking  with  a 
percussion  hammer  the  dorsal  base  of  the  great  toe  (Throckmorton)  or 
by  a  light  scratch  with  a  pin  below  the  external  malleolus  (Chadwick), 
finally  by  pinching  the  tendo  Achillis  (Shaf  er) . 

During  the  same  flaccid  phase  of  hemiplegia  the  face  also  shows 
involvement.  The  paralysis  affects  only  the  lower  half  of  the  face;  the 
angle  of  the  mouth  is  lowered,  the  cheek  on  the  same  side  is  flaccid  and  is 
raised  at  each  expiration,  the  naso-labial  fold  is  smoothened.  The 
asymmetry  of  the  face  is  particularly  noticeable  when  the  patient  speaks 
or  laughs.  The  tongue  protrudes  toward  the  paralyzed  side.  The 
integrity  of  the  upper  branch  of  the  facial  nerve  can  be  seen  from  the 
normal  function  of  the  musculus  frontalis  and  m.  orbicularis  palpebrarum. 
The  reason  of  a  partial  involvement  of  the  face  is  not  very  well  known; 
the  following  possibilities  are  given  by  various  writers:  (1)  different 
course  of  the  fibers  of  the  lower  and  of  the  upper  portions  of  the  facial 
nerve;   (2)  bilateral  innervation  of  the  orbicularis  palpebrarum  muscle 


92 


APOPLEXY 


(Broadbent).  The  neck  and  the  trunk  are  only  very  slightly  involved 
because  of  innervation  of  their  muscles  by  both  hemispheres.  Bilaterally 
associated  movements  are  controlled  by  the  motor  cortex  of  both  hemi- 
spheres of  the  brain.  This  is  seen  from  the  inability  to  move  voluntarily 
one  side  of  the  chest  without  moving  the  other  or  one  side  of  the  forehead 
without  the  other. 

When  the  symptoms  of  paralysis  do  not  dis- 
appear, the  affected  portions  of  the  body  gradually 
enter  into  a  phase  of  secondary  contractures.  It 
is  characterized  by  an  accentuation  of  all  the 
preceding  symptoms.  The  rigidity  of  the  muscles 
becomes  pronounced,  especially  in  the  flexors,  and 
this  is  seen  in  the  upper  as  well  as  in  the  lower  ex- 
tremities and  in  the  face.  Hence  deformities  of 
the  extremities  are  frequently  observed;  they  will 
be  various  according  to  whether  the  flexor  or  ex- 
tensor groups  of  muscles  are  affected.  As  to  the 
various  reflexes,  they  become  more  evident  when 
the  contracture  of  the  muscles  is  moderate,  but 
when  the  latter  is  pronounced,  all  the  reflexes 
may  be  entirely  absent,  viz.,  not  obtainable.  The 
attitude  and  gait  of  hemiplegics  are  quite  charac- 
teristic. The  position  of  the  contractured  upper 
extremity  in  flexion  at  the  elbow,  the  scraping  of 
the  floor  with  the  paralyzed  foot,  the  bending  of 
the  trunk  toward  the  normal  side  in  walking  are 
some  of  the  typical  features  of  hemiplegic  persons 
(Fig.  64). 

The  opposite  side  of  the  body  presents  also  some 
peculiarities.     Exaltation  of  all  deep  reflexes,  some 
Fig.  64.— Attitude  in  weakness  of  muscular  power,  the  presence  of  Bab- 
Hemiplegia  showing  also  inski's  sign  and  of   the   paradoxical  flexor  reflex 

the  Position  of  Contrac-  and  occasionaliy  ankle-Clonus- these  are  the  symp- 
tured  Left  Arm  and  Leg.  j  ,  J      r 

Right  Hempilegia.  toms  observed  on  the  "sound     side  in  some  cases 

of   hemiplegia.     The  reason   of  it  lies  in  the  fact 

that   sometimes  the   homolateral   tract  (see  Anatomy)  is  involved  and 

sometimes  there  is  a  bilateral  lesion  in  the  hemispheres. 

In  recent  years  the  study  of  organic  hemiplegia  became  enriched  by  a 
number  of  new  symptoms  which  in  doubtful  cases  may  render  considerable 
assistance.     They  are  as  follows: 

(1)  The  sign  of  the  interossei  (Souques).     It  consists  of  extension 


APOPLEXY  93 

and    abduction   in  a   fan-like  form    of    the   fingers,  when    the   patient 
attempts  to  raise  the  paralyzed  arm. 

(2)  The  sign  of  the  thumb  (Klippel  and  Weil).  It  consists  of  invol- 
untary flexion  of  the  thumb  when  an  effort  is  made  to  extend  slowly  the 
four  other  fingers  which  are  in  a  state  of  contraction  in  the  hemiplegic 
hand. 

Signs  (1)  and  (2)  are  met  with  only  in  cases  of  hemiplegia  with  con- 
tracture of  the  upper  extremity.  The  following  sign  can  be  demon- 
strated only  in  flaccid  organic  hemiplegia. 

(3)  The  sign  of  the  hand  (Raimiste).  The  elbow  of  the  paralyzed 
arm  is  on  a  table.  The  forearm  and  hand  are  both  raised  and  kept  in  a 
vertical  position  by  the  observer's  hand.  The  latter  is  gently  removed 
from  the  patient's  hand  and  slides  down  on  the  forearm;  the  paralyzed 
hand  at  once  falls  and  forms  an  angle  with  the  forearm  of  1300.  This 
sign  can  be  detected  immediately  after  an  apoplectic  stroke  and  during 
coma. 

(4)  Exaggerated  flexion  of  the  forearm.  When  the  supinated  fore- 
arm is  elevated  and  flexed  over  the  arm,  the  degree  of  flexion  is  greater 
than  in  a  normal  arm  when  the  movement  is  persisted  in. 

(5)  The  finger  phenomenon  (A.  Gordon).  It  consists  of  extension 
of  all  the  fingers,  or  of  the  thumb  and  index,  when  pressure  is  produced 
by  the  observer's  thumb  against  the  pisiform  body.  This  sign  is  particu- 
larly prompt  in  recent  hemiplegias. 

(6)  Chaddock's  "wrist  sign"  consists  of  flexion  of  the  wrist  and  simul- 
taneous extension  and  separation  of  all  the  fingers,  when  scratching 
with  a  pointed  instrument  is  done  on  the  ulnar  side  at  the  junction  of 
the  palm  and  the  wrist. 

(7)  Adduction  and  abduction  sign  of  the  leg  (Raimiste).  When 
the  patient  lying  flat  on  his  back  is  told  to  bring  the  sound  leg  close  to  the 
paralyzed  one  and  at  the  same  time  the  movement  is  resisted  by  the 
observer,  the  paralyzed  leg  instead  makes  an  involuntary  adduction 
movement.  An  abduction  of  paralyzed  limb  is  noticed  when  the  patient 
is  told  to  abduct  his  sound  leg  and  at  the  same  time  opposition  is  made  by 
the  observer. 

(8)  Hypertonicity  of  flexors  sign  (Neri).  When  in  a  lying  position 
the  paralyzed  leg  is  raised,  the  angle  it  forms  with  the  bed  is  but  400, 
while  the  angle  formed  with  the  sound  leg  and  bed  is  700.  When  stand- 
ing patient  is  told  to  bend  his  trunk  forward,  the  sound  leg  will  remain 
straight,  but  the  paralyzed  leg  will  flex. 

(9)  Sign  of  Grasset  and  Gaussel.     When  a  hemiplegic  lying  on  his 


94  APOPLEXY 

back  attempts  to  raise  his  paralyzed  leg,  it  falls  heavily  back  at  the 
moment  the  sound  leg  is  elevated  by  another  person. 

(10)  Babinski's  associated  movements.  When  lying  on  his  back  the 
hemiplegic  attempts  to  raise  himself  without  support  of  his  hands,  the 
paralyzed  leg  extends  and  the  entire  extremity  is  raised  off  the  bed. 

(i.i)  Claude's  reflex  hyperkinesia  is  very  important  from  a  prognostic 
standpoint.  It  consists  of  voluntary  movements  of  a  totally  paralyzed 
limb  when  it  is  placed  in  a  forced  attitude  of  pronation  or  supination  for 
the  upper  extremity;  for  the  lower  extremity  when  the  skin  of  the  external 
aspect  of  the  thigh  is  pinched.  When  this  sign  is  present,  the  prognosis  is 
very  favorable. 

(i  2)  Marie  and  Foix'  retraction  sign.  When  the  toes  are  forced  slowly 
downward,  the  thigh  flexes  over  the  pelvis,  the  leg  over  the  thigh,  and  the 
foot  assumes  a  position  of  adduction. 

(13)  Anticus  leg  sign  (Piotrowski) .  It  consists  of  reflex  dorsal 
flexion  together  with  supination  of  the  foot,  when  the  tibialis  anticus 
muscle  is  percussed  at  its  point  of  origin  between  the  tuberosity  of  the 
tibia  or  head  of  the  fibula  or  else  at  its  belly.  It  is  pathognomonic  of 
organic  diseases  of  the  central  nervous  system,  if  the  reflex  response  is 
excessive  or  when  it  can  be  elicited  only  or  is  predominant  on  one  side. 

Sensory,  Motor  and  Trophic  Complications  of  Hemiplegia 

Sensory. — In  the  majority  of  cases  there  are  some  sensory  disturbances 
on  the  hemiplegic  side.  Numbness,  tingling  or  even  pain  may  precede  or 
follow  the  attack  of  paralysis,  but  the  most  important  sensory  symptom 
is  hemianesthesia.  The  subject  is  of  great  importance,  from  a  diagnostic 
standpoint,  as  the  same  phenomenon  may  be  observed  also  in  hysteria. 
When  the  paralyzed  side  is  carefully  examined,  some  diminution  of  sensa- 
tions to  all  forms  will  almost  always  be  observed.  Sometimes  the  loss 
of  sensations  is  absolute  or  in  proportion  with  the  loss  of  motor  power. 
As  to  the  special  senses,  their  involvement  has  not  always  been  observed. 
Although  the  question  of  disturbed  sensations  in  hemiplegia  is  not  yet 
entirely  settled,  nevertheless  it  is  admitted  by  the  majority  of  writers 
that  while  complete  hemianesthesia  is  rare,  partial  loss  of  sensations  is 
common.  There  are  also  cases  in  which  sensory  disturbances  are  only 
transitory,  and  most  of  the  time  they  develop  shortly  after  the  onset 
of  hemiplegia.  The  characteristic  feature  of  sensory  disturbances  [of 
cerebral  origin  (cortical  or  capsular)  is  their  predominance  in  the  distal 
ends  of  the  limbs.  The  existence  of  partial  or  complete  hemianesthesia 
on  the  paralyzed  side  of  hemiplegics  finds  its  explanation  in  the  anatomical 


APOPLEXY  95 

conception  of  sensory  centers.  (See  Anatomy.)  The  posterior  limb  of 
the  internal  capsule  was  according  to  the  old  teaching  divided  into  two 
portions:  the  anterior  two- thirds  were  exclusively  motor  and  the  posterior 
third  exclusively  sensory  ("carrefour  sensitif "  of  Charcot).  We  are  now 
in  possession  of  a  considerable  number  of  pathological  data  showing  that 
sensory  disturbances  have  been  observed  in  hemiplegia  caused  by  diseased 
foci  in  the  anterior  two-thirds  of  the  posterior  limb  of  the  internal  capsule, 
also  motor  disturbances  as  well  as  sensory  in  hemiplegia  caused  by  a  lesion 
in  the  posterior  third  of  the  same  limb  of  the  capsule.  On  the  other  hand 
there  are  a  few  isolated  facts  which  are  apt  to  throw  some  doubt  on  this 
conception  of  sensory  and  motor  localization.  The  involvement  of 
special  senses  was  found  to  be  present  in  hemiplegias  of  capsular  origin, 
but  not  of  cortical;  however  their  involvement  is  not  as  frequent  as  that 
of  general  sensations.  Special  mention  should  be  made  of  the  stereog- 
nostic  sense.  This  name  is  applied  to  the  perception  of  form  and 
of  physical  properties  of  objects  by  means  of  touch.  In  hemiplegia 
alongside  with  general  sensory  disturbances  astereognosis  is  also  not 
infrequently  observed.  The  writer  had  examined  at  the  Philadelphia 
Hospital  thirty-five  cases  of  motor  cerebral  paralysis  and  found  the 
stereognostic  sense  disturbed  in  twenty-nine  cases,  in  twenty- two  of 
which  the  loss  was  complete  {Journal  of  Nervous  and  Mental  Disease, 

i9°3)  • 

Motor  Complications. — Hemichorea,   hemiathetosis   and   hemiataxia 

(frequently  associated  with  hemianesthesia)  accompany  sometimes 
hemiplegia.  In  the  first  the  paralyzed  arm  and  leg  are  animated  with 
choreiform  movements.  Athetosis  affects  the  fingers  and  toes  and  occa- 
sionally the  muscles  of  the  face.  Hemiataxia  consists  of  incoordination  of 
the  hemiplegic  arm  and  leg  during  voluntary  movements.  The  three  con- 
ditions make  their  appearance  some  time  after  the  onset  of  the  paralysis, 
although  they  may  precede  it.  According  to  Raymond,  these  motor 
symptoms  are  due  to  a  lesion  in  the  internal  capsule. 

Trophic  Complications. — (Edema,  desquamation  of  the  skin,  ulcera- 
tions, disturbed  nutrition  of  the  nails  are  not  infrequent  occurrences  in 
hemiplegias.  Glossy  skin  of  the  hand  is  not  rare.  Among  the  very  fre- 
quent trophic  disturbances  is  muscular  atrophy  of  the  paralyzed  limbs. 

The  atrophy  is  diffuse  affecting  particularly  the  small  muscles  of  the 
hands  and  feet,  also  the  roots  of  the  limbs  viz.  the  humero-scapular  and  the 
gluteal-ferr.oral  regions.  It  may  develop  early,  but  most  frequently  long 
after  the  onset  of  hemiplegia.  There  are  usually  no  reactions  of  degenera- 
tion. In  advanced  cases  the  galvanic  and  faradic  contractility  is  dimin- 
ished.    The  atrophy  is  not  due  to  the  misuse  caused  by  protracted  impo- 


o6  APOPLEXY 

tence,  but  it  is,  according  to  the  generally  accepted  opinion,  of  central 
origin;  it  depends  either  on  a  lesion  of  the  cells  of  the  anterior  cornua  of  the 
spinal  cord,  or  on  the  original  cerebral  lesion.  The  pathogenesis,  how- 
ever, is  not  completely  established.  Very  exceptionally  hypertrophy  of 
the  paralyzed  muscles  is  observed.  Arthropathies  are  sometimes  ob- 
served in  the  shoulders,  ringers,  elbow  or  knee. 

Other  Complications. — In  right-sided  hemiplegias  there  may  be  a 
temporary  or  permanent  loss  of  speech  (aphasia).  The  latter  may  be 
complete  or  partial.  This  subject  is  of  great  importance  and  deserves  a 
special  description,  which  will  be  given  in  a  special  chapter.  Impair- 
ment of  intelligence,  change  of  disposition,  modification  of  character  are 
not  infrequently  observed  some  time  after  the  onset  of  hemiplegia,  espe- 
cially in  people  of  advanced  age. 

Prognosis. — During  the  apoplectic  attack  it  is  almost  impossible  to 
tell  whether  the  patient  will  recover  or  not.  If  the  coma  is  prolonged 
above  twenty-four  or  forty-eight  hours,  the  outlook  is  grave.  It  is  equally 
bad  when  the  bodily  temperature  goes  down  or  ascends  rapidly.  It 
is  also  serious  when  the  high  temperature  is  continuous.  A  cardiac  or 
vascular  disease  renders  also  the  prognosis  uncertain,  as  repetitions  of 
apoplectic  attacks  are  to  be  feared.  Convulsions  and  early  contrac- 
tures are  an  indication  of  a  profuse  hemorrhage  in  the  ventricles  or  in  the 
meninges.  Cheyne-Stokes  respiration  is  of  grave  omen.  Bed-sores  are 
also  an  unfavorable  sign.  The  presence  of  blood  in  the  cerebro-spinal 
fluid  warns  of  impending  danger  even  if  the  clinical  picture  is  at  first  mild. 
When  the  patient  recovers  from  the  coma,  the  question  is  to  determine 
the  future  of  the  paralytic  symptoms.  An  incomplete  hemiplegia  presents 
a  favorable  prognosis.  However  if  the  paralysis  is  at  first  slight,  but 
during  the  following  weeks  becomes  more  pronounced,  the  chances  for 
regaining  the  power  in  the  affected  limbs  are  slight.  Appearance  of  con- 
tractures makes  the  prognosis  serious.  Hemiplegia  due  to  traumatism 
or  syphilis  presents  the  best  prognosis,  as  it  is  amenable  to  treatment. 
;  Complications — sensory,  motor  or  mental,  also  trophic  disturbances, 
convulsions — all  make  the  prognosis  unfavorable. 

Diagnosis.- — Generally  speaking,  there  is  no  difficulty  in  diagnosticat- 
ing^ an  apoplectic  seizure.  In  exceptional  cases,  however,  it  may  be 
confounded  with  uremic  and  diabetic  coma,  syncope,  intoxications 
(chloroform,  alcohol,  lead,  etc.)  and  hysterical  paroxysms,  also  hemor- 
rhagic meningitis.  In  uremia  the  onset  of  the  coma  is  usually  slow  and 
preceded  by  vomiting,  dyspnoea,  convulsions  and  sometimes  visual  dis- 
turbances. In  diabetic  coma  there  is  a  difficult  and  deep  breathing 
followed  by  abrupt  expiration,  vomiting,  diarrhoea;  the  breath  has  the 


APOPLEXY 


97 


odor  of  chloroform  and  sugar  is  found  in  the  urine.  In  syncope  conscious- 
ness is  generally  not  totally  lost,  the  pulse  is  small  and  the  attack  lasts 
but  a  short  time.  The  breath  of  the  patient  will  reveal  an  intoxication, 
although  hemorrhage  in  the  brain  is  not  an  infrequent  occurrence  in  acute 
alcoholism.  A  hysterical  paroxysm  may  simulate  an  apoplectic  attack, 
but  the  facial  expression  showing  emotion,  the  preservation  of  the  corneal 
reflexes  and  the  facility  with  which  the  intensity  of  the  coma  is  modified 
will  enable  one  to  make  the  diagnosis  of  a  hysterical  attack.  Hemorrhagic 
meningitis  accompanied  by  coma  will  be  distinguished  from  apoplectic 
coma  by  the  presence  of  convulsions,  by  absence  of  focal  symptoms  and 
rapid  fatal  termination.  Lumbar  puncture  will  assist  in  determining  the 
etiology  of  coma.  In  cases  of  meningeal  involvement  lymphocytosis  is 
found;  in  diabetic  coma  acetone,  in  uremic  coma  excess  of  urea,  in  lead 
intoxication  high  pressure  of  cerebro-spinal  fluid.  In  a  hysterical 
paroxysm  sudden  loss  of  consciousness  may  supervene,  but  the  respi- 
ration and  pulse  remain  normal;  the  face  is  not  congested.  Besides,  it 
usually  occurs  after  an  emotion. 

After  the  diagnosis  of  apoplexy  is  established,  the  main  problem  con- 
sists in  determining  the  cause  and  the  seat  of  the  lesion. 

In  the  majority  of  cases  it  is  difficult  to  tell  whether  the  apoplexy  is 
due  to  a  hemorrhage  or  softening.  In  favor  of  the  first  there  is  usually 
absence  of  premonitory  symptoms,  its  occurrence  at  a  comparatively 
advanced  age  (above  forty),  low  temperature,  profound  and  prolonged 
coma,  completeness  of  the  paralysis,  redness  of  the  face,  high  tension  of 
the  pulse  and  strong  heart  beats.  A  rapid  and  progressive  improvement 
soon  after  the  onset  is  also  a  characteristic  feature  of  hemorrhage.  In 
favor  of  softening  there  is  usually  a  rise  of  temperature,  absence  of  coma 
or  mild  and  not  prolonged  coma,  incompleteness  of  hemiplegia  (usually  a 
monoplegia) ,  finally  the  presence  of  cardiac  or  arterial  lesions. 

Convulsions  at  the  onset  are  more  frequent  in  softening  than  in 
hemorrhage,  especially  if  they  are  unilateral  and  when  the  cortex  is  affected. 
Early  convulsions  and  early  contractures  are  indicative  of  a  hemorrhage 
in  the  ventricles  or  in  the  meninges  (see  Meningitis).  Aphasia,  if  it  occurs 
with  a  mild  hemiplegia,  is  almost  always  due  to  a  softening.  Persistent 
aphasia  is  caused  by  softening.  Hemianopsia  suggests  softening.  Repeti- 
tion of  apoplectic  attacks  occurs  in  softening.  If  all  these  symptoms 
are  not  sufficient  to  differentiate  hemorrhage  from  softening,  lumbar 
puncture  will  render  assistance.  In  hemorrhage  the  effused  blood  passes 
into  the  cerebro-spinal  fluid  (especially  in  ventricular  hemorrhages)  and 
can  be  detected  by  lumbar  puncture,  while  in  thrombosis  this  is  not  the 
case. 
7 


98  APOPLEXY 

The  next  step  is  to  determine  whether  the  softening  is  due  to  embolism 
or  thrombosis.  A  sudden  onset  without  premonitory  symptoms  in  youth 
and  a  cardiac  lesion  are  symptoms  of  embolism.  In  ulcerative  endocardi- 
tis the  embolism  may  be  followed  by  rise  of  temperature  (chills  and  fever). 
Thrombosis  is  often  preceded  by  paresthesias  in  the  limbs  about  to  be 
paralyzed,  vertigo,  headache,  brief  impairment  of  speech;  the  apoplectic 
insult  comes  on  gradually:  at  first  only  weakness  and  then  a  progressive 
paralysis  without  loss  of  consciousness;  the  hemiplegia  is  usually  incom- 
plete. Atheromatous  condition  of  the  blood  vessels  with  high  blood  pres- 
sure and  old  age  are  found  in  softening  due  to  thrombosis. 

The  above  mentioned  differential  signs  may  be  sufficient  to  determine 
the  nature  of  any  given  case  of  apoplexy.  When  the  hemiplegia  following 
apoplexy  is  definitely  established  and  the  patient  falls  under  observation 
some  time  after  the  insult,  it  is  important  to  ascertain  whether  the 
paralysis  is  of  organic  origin  or  functional.  In  hysterical  hemiplegia  the 
paralysis  is  usually  slow  and  progressive,  is  not  always  confined  strictly  to 
the  entire  half  of  the  body;  one  limb  or  a  part  of  a  limb  is  usually  involved; 
the  face  is  most  frequently  not  involved  and  if  asymmetry  of  the  face 
is  occasionally  observed,  it  is  due  to  a  spasm  but  not  to  a  paralysis.  The 
reflexes  are  normal  (no  Babinski  sign,  no  ankle-clonus,  no  paradoxical 
flexor  reflex  or  any  other  tendon  or  cutaneous  reflex  observed  in  organic 
hemiplegia).  The  course  of  the  disease  is  irregular:  the  paralysis  may 
become  ameliorated  or  aggravated,  alternate  in  its  intensity,  even  disap- 
pear and  reappear;  finally  the  paralysis  may  remain  flaccid  for  an  indefi- 
nite period  of  time.  Spasticity  does  not  succeed  the  early  flaccidity 
which  is  observed  in  organic  cases.  The  gait  is  quite  characteristic.  In 
hysterical  hemiplegia  the  patient  drags  his  leg,  while  in  organic  hemi- 
plegia the  front  part  of  the  foot  sweeps  the  ground  with  each  step.  In 
hysteria  there  is  frequently  a  hemianesthesia  on  the  hemiplegic  side. 
The  latter,  if  present,  is  absolute  and  more  marked  than  the  paralysis, 
while  in  organic  (capsular  or  cortical)  hemianesthesia  the  sensory  dis- 
turbance is  not  evenly  distributed:  the  extremities  are  more  affected  than 
the  trunk  and  the  upper  extremities  more  than  the  lower.  Cerebral 
hemianesthesia  is  usually  transitory  and  it  may  last  but  a  few  hours  (see 
Hysteria). 

Localization. — When  the  lesion  is  in  the  cortex,  the  paralysis  is  fre- 
quently either  in  the  face  and  arm,  or  in  arm  and  leg;  it  is  accompanied 
by  an  incomplete  or  transient  hemianesthesia,  frequently  by  aphasia,  and 
follows  frequently  an  attack  of  Jacksonian  epilepsy. 

Hemiplegia  which  accompanies  a  meningeal  lesion  is  rarely  complete 
and  presents  early  contractures  and  convulsions. 


APOPLEXY  99 

When  the  lesion  is  in  the  internal  capsule,  the  hemiplegia  is  total  and  is 
frequently  accompanied  by  hemiathetosis  or  hemichorea  and  hemianes- 
thesia. 

When  the  hemiplegia  is  due  to  a  hemorrhage  in  the  ventricles,  con- 
vulsions occur  early  and  are  generalized;  there  is  a  progressive  rise  of 
temperature  and  death  is  the  usual  outcome.  Hemiplegia  caused  by 
lesions  in  other  portions  of  the  brain  will  be  described  in  their  respective 
chapters. 

Treatment. — It  was  mentioned  above  that  in  the  majority  of  cases  it 
is  difficult  to  determine  the  cause  of  an  apoplectic  seizure.  In  such  cases 
the  following  measures  should  be  applied.  At  once  the  patient  must  be 
put  to  bed,  all  clothing  loosened,  especially  at  the  neck,  and  a  free  access 
of  fresh  air  given.  He  must  not  be  disturbed  in  the  coma.  The  pulse 
and  heart  should  be  watched  and  according  to  their  condition  stimulants 
or  sedatives  will  be  administered  (hypodermatically  preferred).  With 
return  of  consciousness  the  patient  is  given  a  fluid  diet,  consisting  mainly 
of  milk,  his  bowels  and  bladder  are  closely  watched  and  hypostatic 
congestion  of  the  lung  will  be  avoided  by  changing  frequently  his 
position. 

When  it  is  possible  to  determine  at  the  outset  the  cause  of  the  apo- 
plexy, the  management  will  somewhat  differ  in  hemorrhage  and  softening. 
When  hemorrhage  occurs,  the  patient's  head  will  be  somewhat  raised 
and  the  body  placed  on  the  back  or  on  the  non-paralyzed  side.  As  there 
is  usually  a  flushed  face  and  a  full,  strong  pulse,  a  bleeding  should  be  re- 
sorted to  without  unnecessary  delay.  In  individuals  with  a  strong  heart 
ten  to  fifteen  ounces  of  blood  can  be  taken.  No  venesection  should  be 
practiced  in  cases  of  cardiac  weakness.  Lowering  of  blood  pressure  can 
be  accomplished  not  only  by  venesection  but  also  by  lumbar  puncture 
and  withdrawal  of  a  moderate  quantity  of  cerebro-spinal  fluid.  Compres- 
sion or  ligation  of  the  internal  carotid  artery  on  the  side  of  the  lesion  can 
be  used  in  extreme  cases  for  arresting  a  hemorrhage  after  other  measures 
have  failed.  Puncture  of  the  brain  has  been  advocated  (Frank e),  but 
this  procedure  is  quite  dangerous.  Ice  applied  to  the  head  may  relieve 
congestion.  As  purging  is  a  valuable  procedure,  two  drops  of  croton  oil 
should  be  placed  on  the  back  of  the  tongue.  No  medication  is  to  be  given 
during  the  comatose  state.  With  return  of  consciousness  the  treatment 
should  be  symptomatic.  Restlessness  and  insomnia  are  combated  by 
bromides,  aconite,  trional,  sulphonal  or  veronal.  Weakness  of  the  heart 
will  be  treated  with  mild  stimulants.  Great  caution  should  be  exercised 
in  using  heart  stimulants.  Coffee,  tea  and  alcohol  should  be  avoided, 
unless  there  are  special  indications.     Great  care  must  be  taken  of  the 


IOO  APOPLEXY 

patient's  skin  to  prevent  bed-sores.     Bladder  and  rectum  must  be  emptied, 
if  there  are  no  voluntary  evacuations. 

In  meningeal  hemorrhage  of  adults  trephining  and  ligation  of  the 
bleeding  blood  vessel  are  indicated. 

Meningeal  hemorrhage  in  the  newly  born  deserves  special  mention. 
On  the  first  suspicion  of  a  hemorrhage  a  lumbar  puncture  should  be 
made.  If  this  fails  to  relieve,  the  anterior  fontanelle  should  be  punc- 
tured. Some  advocate  puncture  of  the  fontanelle  at  once  before  doing 
a  lumbar  puncture.  Giles  {Revue  mens.  d'Obstetr.,  Gynec.  et  Pedi- 
atries 191 2)  advises  introduction  of  Pravaz  needle  to  a  depth  of  5  mm. 
or  even  8,  as  far  from  the  longitudinal  sinus  as  possible  in  the  fronto- 
parietal angle  of  the  fontanelle.  This  draws  a  certain  amount  of  blood 
at  once  before  it  has  had  the  time  to  clot  and  the  rest  is  easily  absorbed. 
Puncture  of  the  fontanelle  is  merely  an  exploratory  operation,  but  it 
may  have  an  actual  curative  value  as  in  a  case  observed  by  Giles  {lot. 
cit.) .  It  is  an  excellent  preparatory  procedure  as  it  gives  an  opportunity 
to  wait  until  the  brain  develops  more  resisting  power  before  a  radical 
operation  is  attempted  to  relieve  pressure  on  the  brain. 

When  the  indications  are  that  the  apoplectic  stroke  is  due  to  an 
embolism  or  thrombosis,  the  patient's  position  should  be  somewhat 
different  from  that  in  hemorrhage:  the  head  must  be  somewhat  lowered 
or  the  entire  body  laid  flat.  Heart  stimulants  must  be  instantly  ad- 
ministered, if  the  weakness  of  the  heart  is  evident  or  when  the  blood 
pressure  is  low:  ether,  camphor  and  nitroglycerine  should  be  administered 
hypodermatically;  wine,  brandy,  tea  and  coffee  by  the  mouth.  Applica- 
tion of  ice  and  venesection,  which  are  sometimes  beneficial  in  cerebral 
hemorrhage,  are  contra-indicated  in  softening.  The  further  treatment 
of  softening  after  the  immediate  symptoms  have  subsided  will  be  symp- 
tomatic. The  condition  of  the  sphincters  and  of  the  skin  must  be  care- 
fully watched.  The  diet  must  consist  mainly  of  milk,  eggs  and  fruit. 
Later  on,  many  weeks  after  the  onset,  when  only  hemiplegia  is  present, 
meats  and  vegetables  may  be  added  to  the  above  diet.  As  internal 
medication  iodides  are  advisable  in  both  softening  and  hemorrhage, 
especially  in  those  cases  in  which  there  are  evidences  of  specific  arteritis. 
In  cases  of  arteriosclerosis  with  a  high  tension  pulse,  occasional  adminis- 
tration of  nitroglycerine  in  gr.  1/100  doses  two  or  three  times  a  day  is 
advisable. 

The  treatment  of  hemiplegia  should  begin  as  soon  as  the  general  con- 
dition permits  it,  and  this  is  usually  in  the  second  week.  Passive  and 
active  movements  in  addition  to  massage  are  the  only  means  of  which 
improvement  can  be  expected.     Massage  is  the  most  important  one. 


APOPLEXY  IOI 

I  have  seen  mild  cases  which  have  so  much  improved  from  early  massage 
that  the  paralysis  was  hardly  noticeable.  It  is  a  valuable  procedure  not 
only  in  recent  but  also  in  old  hemiplegias  with  contractures.  The  mas- 
sage must  be  given  as  often  as  possible — every  day  and  even  twice  a  day. 
It  prevents,  when  instituted  early,  the  rapid  tendency  to  contractures 
by  improving  the  nutrition  of  the  muscles  and  their  tendons;  it  prevents 
ankylosis  of  the  joints  and  removes  the  paresthesias  which  so  frequently 
accompany  the  paralysis.  Systematic  re-education  of  movements  of 
the  affected  limbs  is  important;  the  patient  must  persist  in  exercising 
them  frequently.  Contractures  may  also  be  treated  by  warm  baths 
followed  by  massage  and  passive  movements.  Electricity,  which  is  so 
frequently  employed  in  organic  nervous  affections,  should  be  avoided 
in  organic  hemiplegias,  as  it  is  apt  to  hasten  or  increase  the  contracture 
of  the  muscles.  For  the  same  reason  the  drug  which  has  a  tendency  to 
increase  the  tonicity  of  the  muscles  should  never  be  given  in  hemiplegia 
— and  this  is  strychnia.  To  combat  the  rigidity  of  the  limbs  in  paralysis 
of  cerebral  origin  Foerster  has  devised  an  operation  on  the  spinal  roots.  It 
will  b.e  discussed  in  the  chapter  on  Diplegia.  See  also  "muscle  isolation" 
method  of  Schwab  and  Allison,  also  that  of  Stoffel  (pages  122  and  123). 
The  complications  of  hemiplegia  do  not  require  any  special  treatment. 
The  hemiathetosis,  hemichorea  and  hemiataxia  may  improve  with  the 
amelioration  of  the  hemiplegia  itself.  The  aphasia  which  occasionally 
occurs  depends  upon  the  gravity  of  the  cerebral  lesion.  However  at- 
tempts of  reeducation  of  speech  have  been  tried  by  some  and  fair  results 
reported.  No  special  rules  can  be  given  for  the  treatment  of  the  mental 
condition  in  hemiplegia. 

INGRAVESCENT  OR  PROGRESSIVE  APOPLEXY 

It  is  characterized  by  sudden  vertigo  and  headache,  rapidly  followed 
by  a  hemiplegia.  There  is  no  loss  of  consciousness.  Gradually  the  pa- 
tient becomes  somnolent,  stuporous,  comatose  and  dies  at  the  end  of  a 
few  days.  The  lesion  consists  of  a  hemorrhage  caused  by  rupture  of  the 
external  lenticular  artery.  The  blood  spreads  forward  and  backward 
and  finally  invades  the  lateral  ventricle. 

DELAYED  APOPLEXY 

Spatapoplexie 

Under  this  name  is  understood  an  attack  of  apoplexy  with  loss  of 
consciousness  followed  by  hemiplegia.     It  occurs  some   time   (but  not 


102  APOPLEXY 

immediately)  after  trauma.  It  was  first  described  by  B dinger  in  1891. 
Cases  have  been  reported  in  which  the  interval  between  the  traumatism 
and  the  stroke  lasts  from  six  days  to  four  weeks.  Autopsy  showed  in  the 
majority  of  cases  a  cerebral  hemorrhage.  Trousseau  has  shown  that  a 
rupture  of  a  blood  vessel  may  occur  some  time  after  a  concussion  by  means 
of  a  thrombosis  of  capillaries;  a  focus  of  softening  is  thus  formed  in  the 
center  of  which  a  secondary  hemorrhage  takes  place.  In  the  cases  of 
delayed  apoplexy  the  blood  vessels  were  found  diseased  (arteriosclerosis). 
The  trauma  therefore  plays  only  an  exciting  role  in  an  individual  with 
an  already  altered  vascular  system.  It  is  well  to  remember  that  nervous 
disorders  occurring  some  time  after  an  accident  are  not  always  func- 
tional; they  may  be  also  organic  and  present  a  grave  prognosis. 

INTERMITTENT  CLOSING  OF  CEREBRAL  ARTERIES  AND  TRANSITORY 

HEMIPLEGIA 

Besides  hemorrhage,  embolism  and  thrombosis  as  causes  of  hemi- 
plegia, a  sudden  closure  of  a  cerebral  blood  vessel  due  to  a  spasm  may 
also  produce  a  paralysis.  Bastian  was  the  first  to  call  attention  to  this 
condition  in  1875.  Not  infrequently  cases  come  under  observation  in 
which  pa  ralysis  comes  on  suddenly  and  disappears  rapidly.  Sometimes 
an  attack  consists  only  of  sensations  of  tingling  or  numbness  in  arm  or 
leg  or  in  both,  which  may  or  may  not  be  followed  by  paralysis  or  paresis 
on  the  same  side.  Sometimes  it  may  be  only  an  attack  of  aphasia  with 
or  without  hemiplegia.  The  chief  characteristic  of  these  attacks  is 
their  suddenness  of  onset  and  of  disappearance,  also  their  reappear- 
ance at  short  or  long  intervals.  Besides,  there  is  no  loss  of  consciousness 
in  the  majority  of  cases. 

The  spasm  of  the  artery  may  produce  either  its  complete  or  incom- 
plete occlusion.  As  a  result  there  is  either  suspension  or  impairment 
of  function.  The  cause  of  the  spasm  lies  probably  in  the  disturbance 
of  the  vaso-motor  nerve  supply  of  the  arteries.  The  existence  of  vaso- 
constrictors has  been  shown  by  C.  F.  Wiggers  (Amer.  J.  of  Phys.,  1905, 
1907,  1908).  Complete  recovery  after  each  of  these  attacks  proves 
that  the  hemiparesis  or  hemiplegia  is  not  due  to  hemorrhage  or  soften- 
ing, but  to  an  irregular  vessel  action  and  therefore  to  an  irregular  blood 
flow  in  the  brain.  If  by  proper  hygienic  measures  the  irritability  of  the 
vascular  tonus  can  be  prevented,  no  further  attacks  will  occur.  Other- 
wise it  facilitates  formation  of  thrombosis.  Recurrent  attacks  may  be 
considered  as  threatening  or  premonitory  signs  of  eventual  cerebral 
hemorrhage  or  thrombosis. 


APOPLEXY  IO3 

The  treament  of  such  cases  consists  of  internal  administration  of  vaso- 
dilators (nitroglycerine),  avoidance  of  emotion,  of  proper  elimination 
(bowels  and  kidneys),  avoidance  of  red  meats.  As  the  disorder  usually 
occurs  in  elderly  persons  showing  evidences  of  arterial  changes,  prolonged 
rest  and  potassium  or  sodium  iodide  should  be  added  to  the  above 
treatment. 


CHAPTER  V 

INFLAMMATION  OF  THE  BRAIN  (ENCEPHALITIS, 
CEREBRITIS).    ACUTE  AND  CHRONIC 

A.  ACUTE  ENCEPHALITIS 

A  primary  acute  inflammation  of  the  brain  tissue  is  a  rare  affection. 
It  is  usually  a  circumscribed  lesion  and  almost  always  leads  to  suppura- 
tion. Trauma  is  considered  as  one  of  the  causes,  but  in  such  cases  the 
meninges  as  well  as  the  brain  tissue  are  commonly  involved,  although  in 
rare  cases  the  former  escape.  The  most  frequent  cause  of  primary  en- 
cephalitis is  infection.  Although  the  organism  is  not  known,  neverthe- 
less recent  experimental  evidences  indicate  resemblance  of  acute  encepha- 
litis to  poliomyelitis.  Both  are  transmissible  diseases  and  probably 
constitute  acute  specific  diseases.  In  the  course  of  infectious  diseases, 
as  diphtheria,  erysipelas,  scarlet  fever,  measles,  acute  tuberculosis,  pneu- 
monia, ulcerative  endocarditis  and  whooping  cough,  encephalitis  may 
develop.  In  these  cases  the  inflammation  may  and  may  not  terminate 
in  suppuration.  Cases  in  which  there  is  always  formation  of  abscess  are 
those  with  a  history  of  caries  of  petrous  bone,  of  frontal  sinuses  and  espe- 
cially with  otitis  media.  The  term  "acute  hemorrhagic  encephalitis" 
is  applied  to  those  forms  of  encephalitis  which  do  not  end  in  suppuration. 
The  two  forms  deserve  special  descriptions. 

(a)  Non-suppurative  Form 

This  variety  of  encephalitis  affects  the  cerebrum  more  frequently 
than  the  cerebellum.  Strumpell  in  1884  was  the  first  to  call  attention 
to  a  primary  cortical  cerebritis  (polio-encephalitis)  in  the  motor  area. 
He  observed  it  in  children,  and  acute  cerebral  palsy  was  due,  in  his  opin- 
ion, to  this  cause.  A  similar  condition  may  also  occur  in  the  pons,  in 
the  gray  substance  around  the  aqueduct  of  Sylvius  and  is  called  then 
"polio-encephalitis  superior  of  Wernicke,"  to  be  distinguished  from 
" polio-encephalitis  inferior"  or  acute  bulbar  paralysis. 

Pathology. — Irrespective  of  the  seat  the  lesion  is  iedntical  in  both 
cases.  The  affected  area  appears  swollen  and  markedly  red;  its  consist- 
ency is  lessened.     The  blood  vessels,  especially  the  capillaries,  are  dis- 

104 


INFLAMMATION    OF    THE   BRAIN  105 

tended,  foci  of  hemorrhage  are  abundant,  nervous  tissue  and  the  blood 
vessels  are  infiltrated  with  leucocytes.  The  brain  tissue — fibers,  gang- 
lion-cells and  neuroglia — undergoes  degeneration.  When  the  nerve  tissue 
is  totally  disintegrated,  it  is  eventually  absorbed;  walls  will  form  around 
the  remaining  cavity  and  a  cyst  will  be  the  result;  the  latter  may  be- 
come contracted  and  a  cicatrix  develops.  Restitution  of  the  affected 
nerve  tissue  is  rare. 

Symptoms.- — The  clinical  manifestations  of  the  non-suppurative 
variety  of  encephalitis  affecting  the  floor  of  the  aqueduct  of  Sylvius 
will  not  be  discussed  here.  In  the  chapter  on  diseases  of  the  medulla 
this  subject  will  be  taken  up  in  detail. 

The  symptoms  of  encephalitis  appear  acutely.  The  onset  resembles 
that  of  acute  infection.  Sudden  rise  of  temperature,  chills,  headache, 
sometimes  vomiting  and  convulsions  are  the  usual  prodromal  signs. 
Some  rigidity  of  the  neck  muscles  is  common.  Loss  of  consciousness  or 
only  incomplete  coma  with  delirium,  Cheyne-Stokes'  respiration  and 
rapid  pulse  are  the  actual  general  symptoms  of  the  affection.  The 
localized  symptoms  will  depend  upon  the  seat  of  the  inflammation  (see 
chapter  on  cerebral  localizations) ,  and  as  in  the  majority  of  cases  the  cortex 
of  the  motor  area  is  involved,  hemiplegia  or  monoplegia  with  local  con- 
vulsions with  or  without  aphasia  are  the  most  frequent  occurrences. 
Acute  encephalitis  attacks  children  oftener  than  adults.  For  this  reason 
mental  arrest  is  observed  in  young  individuals  who  had  once  an  acute 
attack  of  cerebritis. 

Prognosis. — During  the  comatose  state  the  prognosis  is  usually 
unfavorable.  Death  may  occur  in  twenty-four  hours.  The  severity 
of  the  prodromal  symptoms  and  the  degree  of  the  coma  generally  deter- 
mine the  outlook  in  a  given  case.  After  the  acute  symptoms  subside, 
the  prognosis  becomes  more  favorable.  Recovery  without  some  defect 
is  rare:  focal  epilepsy,  impaired  speech,  some  paralytic  condition  of  one 
or  two  limbs  on  the  same  side  of  the  body  are  the  most  frequent  sequelae 
of  acute  encephalitis. 

Diagnosis. — Meningitis  and  cerebral  hemorrhage  are  the  two  affec- 
tions with  which  acute  encephalitis  may  be  confounded.  When  focal 
symptoms,  such  as  described  above,  make  their  appearance  at  the  onset 
and  become  rapidly  more  and  more  pronounced,  encephalitis  should  be 
suspected  rather  than  a  localized  meningitis.  For  the  differential  diag- 
nosis with  cerebral  hemorrhage  see  chapter  on  Apoplexy. 

Treatment.- — During  the  acute  stage  application  of  ice  to  the  head 
and  bleeding,  in  addition  to  absolute  rest,  are  practically  the  only  meas- 
ures that  could  be  recommended.     If  the  fever  is  very  high  antipyretics 


106  INFLAMMATION    OF    THE   BRAIN 

should  be  used.     The  treatment  of  the  chronic  stage  is  identical  with  that 
of  apoplexy. 

(b)  Suppurative  Form.    Abscess  of  the  Brain 

Etiology. — As  mentioned  above,  the  most  constant  cause  of  acute 
encephalitis  terminating  in  suppuration  is  a  lesion  of  the  bony  walls  of  the 
cranium.  Trauma  of  the  scalp,  of  the  meninges  and  of  the  brain  tissue 
may  also  be  followed  by  formation  of  an  abscess.  In  connection  with 
this  factor  it  should  be  borne  in  mind  that  in  some  cases  abscesses  of  the 
brain  were  observed  long  after  the  occurrence  of  the  trauma,  also  that  in 
a  certain  group  of  cases  the  trauma  of  the  head  was  slight  and  still  suppura- 
tion developed  in  the  brain.  A  metastatic  embolus  originating  in  some 
purulent  focus  of  the  body,  like  cellulitis,  abscess  of  the  liver,  purulent 
processes  in  the  bronchi  or  lungs,  or  other  viscera,  is  another  cause  of 
abscess  of  the  brain.  Suppuration  of  the  mucous  membranes  lining  the 
cavities  of  the  cranium  (nose  and  accessory  cavities)  is  the  third  cause  of 
abscess.  Finally  the  most  important  and,  according  to  some  observers, 
the  most  frequent  cause  of  abscess  of  the  brain  is  Otitis  media  and  espe- 
cially its  chronic  form.  Small  veins  and  lymphatics  pass  from  the  tym- 
panum to  the  superior  petrosal  sinus;  similar  vessels  end  in  the  same 
sinus  from  the  temporo-sphenoidal  lobe  of  the  brain.  Identical  vessels 
come  from  the  lateral  lobe  of  the  cerebellum  and  from  the  mastoid  region 
and  all  end  in  the  lateral  sinus.  The  channels  of  infection  are  thus  ex- 
plained. Diseases  of  the  ear  are  apt  therefore  to  produce  an  abscess  in 
the  temporo-sphenoidal  lobe  and  in  the  lateral  lobe  of  the  cerebellum. 

In  cases  of  abscess  caused  by  diseases  of  the  nasal  cavity  the  abscess 
is  situated  in  the  frontal  lobe.  The  infection  is  carried  through  the 
venous  and  lymphatic  channels  or  directly  from  the  carious  bone. 

Whatever  the  original  cause  may  be,  suppuration  in  the  brain  neces- 
sitates the  presence  of  multiple  microorganisms,  among  which  the  strep- 
tococcus plays  the  most  important  role.  An  interesting  and  rare  variety 
is  the  cerebral  suppuration  in  which  the  tubercle  bacillus  alone  was  found 
(Fraenkel,  Rendu  and  Boulloche). 

The  infection  may  be  transmitted  to  the  brain  tissue  either  through 
the  venous  sinuses,  which  are  then  in  a  state  of  inflammation,  or  through 
the  arterial  system.  The  latter  will  occur  in  those  cases  in  which  the 
initial  purulent  focus  is  far  away  from  the  brain;  the  former  will  take  p]ace 
when  the  suppuration  is  in  proximity  of  the  brain. 

As  to  the  seat  of  abscess,  it  is  most  frequent  in  the  hemispheres,  rarely 
in  the  basal  ganglia;  the  metastatic  form  is  in  the  area  of  distribution 


INFLAMMATION    OF    THE   BRAIN 


107 


of  the  middle  meningeal  artery;  the  otitic  form  has  its  seat  in  the  temporal 
lobe  or  cerebellum;  the  traumatic  form  is  usually  confined  to  the  injured 
region  of  the  cranium.  The  average  age  at  which  cerebral  abscess  is  met 
with  is  between  twenty-five  and  forty;  it  is  rare  above  sixty.  Males 
are  more  frequently  affected  than  females. 

Pathology. — There  are  two  principal  conditions  to  be  considered: 
one  in  which  there  is  a  purulent  infiltration  with  subsequent  softening 
of  the  involved  cerebral  tissue,  the  other  is  characterized  by  formation 
of  an  abscess  which  becomes  encapsulated.  In  the  latter  case  the  mem- 
brane surrounding  the  purulent  cavity  is  organized  by  the  neuroglia 
tissue;  its  formation  commences  on  the  fifteenth  day.  If  the  capsule 
is  complete,  it  serves  as  a  protection  for  the  surrounding  nervous  tissue 
against  invasion  of  the  pus,  but  not  infrequently  fistulous  channels  are 


Fig.  65. — Hemorrhage  in  the  Prefrontal  Lobe  Following  Trauma.     (Original.) 

to  be  found  and  formation  of  pus  continues  indefinitely.  As  to  the  capsule 
itself,  it  is  thin  at  the  beginning;  later  it  grows  thick  and  calcification 
may  also  occur.  While  it  is  thin  and  delicate  and  the  pus  continues  to 
accumulate,  it  is  apt  to  rupture.  When  the  latter  occurs  on  the  surface 
of  the  brain  a  purulent  meningitis  will  be  the  result.  When  the  pus 
breaks  through  the  capsule  into  the  ventricles  sudden  death  may  follow. 
Single  encapsulated  abscesses  are  met  with  in  the  majority  of  cases, 
but  multiple  abscesses  may  also  occur,  especially  in  the  metastatic  form 
and  in  general  pyemia.  The  brain  tissue  in  both  cases,  namely  in  purulent 
infiltration  and  abscess,  undergoes  at  first  a  so-called  "  red  softening  " 
in  which  disintegrated  nerve  elements,  leucocytes  and  microorganisms 
are  found.     The  leucocytes  become  gradually  but  rapidly  more  abundant. 


108  INFLAMMATION   OF   THE  BRAIN 

They  surround  the  blood  vessels,  and  cover  the  nerve  tissue,  which  is  in 
a  state  of  inflammatory  oedema.  Cells  and  nerve  fibers  are  gradually 
destroyed  and  replaced  by  pus.  Softening  of  cerebral  tissue  is  observed 
also  in  the  immediate  neighborhood  around  an  encapsulated  abscess  (Figs. 
65  and  66).  Cerebral  abscesses  are  more  frequent  than  cerebellar.  In 
the  brain  abscesses  are  mostly  found  in  the  temporo-sphenoidal  lobe. 
In  the  cerebellum  in  its  lateral  lobe.  Abscesses  are  rare  at  the  base  of 
the  brain. 

Symptoms. — The  clinical  manifestations  are  general  and  local. 


.'.  ..  0 


CV 

Fig.  66. — Hemorrhagic  Encephalitis  in  Cortex.     {From  Flatan,  Jacobsohn,  Minor.) 

In  center  a  blood  vessel  with  round-cell  infiltration,     d.  Focus  of  round  cells,     c.  Amorphous 

exudate,     a.  Fresh  blood,     b.  Mass  of  round  cells. 

(a)  General. — At  the  onset  the  symptoms  are  those  of  inflammation. 
Fever  is  not  pronounced,  but  what  is  often  observed  is  the  lack  of  paral- 
lelism between  the  pulse  and  fever;  while  the  latter  is  high,  the  pulse 
may  be  slow.  The  fever  is  not  infrequently  accompanied  by  delirium 
and  occasionally  by  generalized  convulsions  especially  when  meningi- 
tis or  sinus  phlebitis  are  associated.  In  the  majority  of  cases  there  is  no 
fever;  the  temperature  is  either  normal  or  sub-normal.  The  pulse  is 
usually  slow;  rapidity  of  pulse  indicates  either  a  complication  of 
sinus  phlebitis  or  rupture  of  the  abscess  into  the  lateral  ventricles. 
Headache  is  among  the  earliest  symptoms,  and  when  it  is  continuous  and 


INFLAMMATION    OF   THE  BRAIN  109 

accompanied  by  fever  it  becomes  pathognomonic  of  acute  encephalitis. 
The  localized  character  of  the  pain  in  the  head  and  its  exacerbations  on 
the  slightest  movement  make  it  quite  typical  of  acute  suppurative  en- 
cephalitis. In  cerebellar  abscess  the  pain  is  mostly  in  the  occipital  region. 
Vomiting  is  quite  common,  especially  in  cerebellar  abscess.  Insomnia 
and  restlessness  are  frequent.  Rigidity  of  the  neck,  photophobia  and 
ocular  palsies  are  sometimes  observed.  Optic  neuritis,  although  not  con- 
stant, has  been  seen  even  early  in  the  acute  stage.  Mental  hebetude 
and  apathy  are  conspicuous. 

Leucocytosis  is  frequent  in  brain  abscess. 

(b)  Local  Symptoms. — They  are  mainly  paralysis  and  focal  epilepti- 
form convulsions.  The  latter  are  less  common  than  the  first.  Hemi- 
plegia or  monoplegia  are  usually  marked,  but  may  be  also  absolute;  they 
generally  follow  a  unilateral  convulsion.  As  an  abscess  may  occur  in 
any  part  of  the  brain,  the  special  symptoms  will  depend  upon  its  seat 
and  are  therefore  similar  to  those  of  tumors.  Thus  motor  and  sensory 
aphasia,  blindness,  hemianopsia,  etc.,  may  be  observed.  They  will  be 
described  in  the  chapter  on  tumors  of  the  brain. 

Special  mention  deserves  cerebellar  abscess,  which  is  not  an  infrequent 
occurrence  in  otitic  cases.  Vertigo  and  staggering  gait  ard  particularly 
pronounced.  The  tendency  to  fall  is  usually  toward  the  side  of  the  abscess. 
As  the  cerebellum  forms  partly  the  roof  of  the  fourth  ventricle,  the  abscess 
may  produce  pressure  on  the  medulla  and  cause  bulbar  symptoms. 

Course  of  the  Disease. — The  onset  of  acute  suppurative  encephalitis 
is  very  much  similar  to  that  of  tubercular  meningitis  in  children.  In 
the  large  majority  of  cases  the  clinical  picture  is  as  follows.  Fever  is  the 
first  symptom  to  appear.  Soon  violent  headache  sets  in.  The  patient 
becomes  restless,  agitated,  cannot  sleep,  refuses  to  take  food,  sometimes 
has  attacks  of  vomiting.  As  the  disease  advances,  he  becomes  delirious, 
is  seized  with  generalized  convulsions;  the  neck  is  rigid;  ocular  palsies 
make  their  appearance.  Then  the  patient  enters  into  a  second  state,  in 
which  all  the  symptoms  become  ameliorated.  This  condition,  however, 
does  not  last  any  marked  length  of  time,  as  it  is  soon  followed  by  a  state 
of  apoplexy.  Here  the  coma  may  be  so  profound  that  the  patient  will 
not  regain  consciousness  and  die.  If  he  does  recover,  rigidity  and  paraly- 
sis will  be  present  on  one  side  of  the  body.  The  further  evolution  of  the 
disease  will  depend  upon  the  anatomical  course  of  the  abscess.  If  the 
abscess  becomes  diffused  or  breaks  into  the  ventricles,  death  will  ensue. 
Some  cases  of  acute  abscess  run  such  a  rapid  course  that  fatal  termina- 
tion may  take  place  in  a  very  short  time.  In  the  course  of  some  cases 
of  cerebral  abscess  associated  with  otorrhea  the  discharge  may  repeat- 


IIO  INFLAMMATION    OF    THE   BRAIN 

edly  stop.  At  each  stoppage  there  is  a  marked  aggravation  of  headache. 
It  is  due  to  increase  of  intracranial  pressure.  The  reestablishment  of 
otorrhea  relieves  the  symptoms.  There  is  a  series  of  cases  with  a  history 
of  trauma  or  of  an  old  otitis,  in  which  the  abscess  remains  latent  for 
months  and  even  years  without  being  suspected,  when  death  takes  place 
either  from  rupture  of  the  abscess  or  from  acutely  developed  morbid 
processes  in  or  in  the  vicinity  of  the  abscess.  This  is  the  so-called  "latent 
form."  To  the  same  group  belongs  those  cases  in  which  the  abscess  is 
seated  in  the  depth  of  the  frontal  lobe  or  in  the  postero-external  portion 
of  the  occipital  lobe.  In  such  a  case  there  are  very  few  symptoms  during 
life;  sudden  death  is  the  usual  result.  However  the  "latent  form"  may 
in  some  cases  end  gradually :  an  intercurrent  acute  meningitis  may  aggra- 
vate the  few  existing  symptoms  which  continue  then  to  increase  until 
death. 

Prognosis. — Generally  speaking  the  outlook  is  grave.  The  difficulty 
of  making  a  diagnosis,  especially  in  the  "latent"  cases,  the  dimculty  of 
localizing,  the  damage  produced  in  the  brain  tissue  by  the  abscess  itself 
or  by  operative  procedures  are  the  reasons  why  abscess  of  the  brain  bears 
a  serious  prognosis.  However  there  is  quite  a  considerable  number  of 
observations  showing  that  immediate  death  can  be  avoided  by  prompt 
surgical  intervention.  Complete  recoveries  have  been  also  reported  by 
some  writers,  especially  in  uncomplicated  cases  and  in  those  that  were 
operated  upon  early.  In  chronic  or  subacute  cases  the  prognosis  is  more 
favorable  than  in  the  acute  ones.  In  the  latent  cases  the  abscess  may 
remain  encapsulated  for  a  long  time  without  producing  serious  dis- 
turbances, but  the  patient  is  always  threatened  with  complications,  such 
as  meningitis  and  cedema  of  the  brain. 

Diagnosis. — When  a  history  of  trauma  is  given,  the  diagnosis  of 
abscess  is  not  difficult.  It  is  also  comparatively  easy  to  diagnosticate 
abscess,  when  in  the  course  of  otitis  media  a  localized  palsy  affecting  one 
or  two  extremities  makes  its  appearance.  In  all  other  cases,  especially  in 
the  "latent  forms"  the  diagnosis  is  extremely  difficult  and  can  be  made 
only  by  exclusion.  Ear,  nose,  orbit  and  other  parts  of  the  skull  should  be 
examined  in  every  case.  A  septic  otorrhea  and  a  small  perforation  of  the 
tympanum  are  in  favor  of  a  cerebral  abscess;  when  the  opening  of  the 
tympanic  membrane  is  large,  the  pus  will  have  a  free  exit.  Absence  of 
perforation  also  speaks  for  a  brain  abscess. 

When  there  is  no  external  evidence  of  injury  or  of  bone  and  ear  disease, 
metastasis  should  be  thought  of.  Heart,  lung  and  other  viscera  must  be 
carefully  examined.  A  cerebral  tumor  may  give  identical  symptoms, 
but  its  course  is  uniformly  progressive  and  by  far  more  prolonged  than 


INFLAMMATION    OF    THE  BRAIN  III 

that  of  abscess.  Moreover  convulsions,  if  they  do  occur,  are  more  frequent 
and  more  regular  than  in  abscess.  Headache,  which  is  of  great  importance 
in  abscess,  is  more  progressive,  severer  and  longer  in  duration  in  tumors. 
Involvement  of  the  cranial  nerves  and  especially  double  optic  neuritis 
and  atrophy  are  common  in  tumor  and  exceptionally  rare  in  abscess.  A 
unilateral  optic  neuritis  is  in  favor  of  abscess.  Sudden  appearance  of 
symptoms  of  meningitis  or  of  sinus  phlebitis  is  an  indication  of  abscess. 
Finally  a  history  of  ear  disease  with  a  long  course  of  brain  symptoms 
points  to  an  abscess,     Leucocytosis  is  also  in  favor  of  abscess. 

Meningitis  can  be  confounded  in  certain  cases  with  abscess,  but  the 
rapid  onset,  involvement  of  cranial  nerves,  high  temperature,  rapid  and 
irregular  pulse  are  all  symptoms  rarely  found  in  abscess.  Lymphocytosis 
and  a  cloudy  appearance  of  the  cerebro-spinal  fluid  are  characteristic  of 
meningitis.  In  children  tuberculous  meningitis  may  be  confounded  with 
cerebral  abscess. .  Rigidity  of  the  neck,  attacks  of  focal  epilepsy  or  of 
paralysis,  the  elevation  of  temperature  and  the  above  condition  of  the 
cerebro-spinal  fluid  are  in  favor  of  tuberculous  meningitis. 

Traumatism  of  the  cranium  is  liable  to  develop  a  hemorrhagic  pachy- 
meningitis. The  distinguishing  symptoms  between  this  form  and 
abscess  consist  of  the  character  of  pain,  which  is  superficial  and  localized 
and  not  augmented  by  motion  in  pachymeningitis,  and  of  the  fever, 
which  is  only  transitory  in  the  latter  affection. 

Sinus  phlebitis  will  be  recognized  by  high  temperature,  rapid  pulse, 
chills,  sweating  and  particularly  by  tenderness  and  swelling  over  the 
origin  of  the  internal  jugular  vein. 

In  cases  of  suspected  abscess  with  otitis  it  is  important  to  know  whether 
the  abscess  is  in  the  temporo-sphenoidal  lobe  or  in  the  cerebellum.  Pro- 
nounced occipital  headache  with  other  symptoms  characteristic  of 
cerebellar  diseases  will  enable  one  to  make  the  differential  diagnosis. 

Treatment. — As  soon  as  strong  suspicions  of  abscess  are  present, 
prompt  surgical  intervention  should  be  the  only  treatment.  In  a  number 
of  cases  it  is  difficult  to  make  a  diagnosis.  In  the  obscure  cases,  in  which 
but  a  few  symptoms  are  evident,  abscess  should  be  thought  of  and  the 
patient  be  treated  by  other  means  only  a  short  time.  Counter-irritation 
and  local  bleeding,  application  of  ice  to  the  painful  region,  purgation, 
sedatives  to  allay  excitement,  stimulants  to  combat  depression  are  the 
usual  means  employed  in  similar  cases.  The  medical  treatment  should  be 
kept  up  for  a  very  brief  period,  and  if  there  is  no  marked  improvement,  an 
operation  must  be  promptly  resorted  to. 

Great  attention  should  always  be  paid  to  cranial  injuries,  viz.  guard 
against  infection,  as  the  treatment  may  be  very  efficacious  in  such  cases 


112  INFLAMMATION   OF   THE  BRAIN 

before  an  abscess  is  formed.  Otitis  media  should  never  be  neglected,  no 
matter  how  slight  it  may  be.  Statistics  show  that  early  surgical  inter- 
vention gave  a  large  percentage  of  recoveries.  When  the  abscess  is 
due  to  diseases  of  the  ear,  the  operation  must  be  performed  over  the  area 
corresponding  to  the  temporo-sphenoidal  lobe  or  to  the  cerebellum.  In 
the  latter  case  there  is  usually  an  occipital  headache,  also  rigidity  of  the 
neck  and  retraction  of  the  head.  Besides,  the  patient  will  present  the 
typical  symptoms  of  a  cerebellar  disease,  viz.  asynergia,  adiadochokinesia, 
hypotonia,  etc.,  etc.  (see  Cerebellar  Diseases).  The  temporo-sphenoidal 
lobe  is  considered  as  a  silent  region  and  there  are  usually  no  localizing 
symptoms,  but  if  the  abscess  encroaches  upon  the  first  and  second  temporal 
lobes,  there  will  be  in  addition  to  the  general  symptoms  of  cerebral  abscess 
also  word-deafness.  For  localizing  symptoms  of  abscess  of  other  portions 
of  the  brain,  see  Cerebral  localizations.  The  surgeon  will  therefore  be 
guided  by  the  above  considerations.  In  the  traumatic  cases  a  careful 
examination  of  the  parts  injured  will  have  to  be  made. 

B.  CHRONIC  ENCEPHALITIS 

Chronic  inflammation  of  the  brain  in  adults  is  very  rare  as  a  primary 
affection.  It  is  encountered  in  isolated  foci  in  the  vicinity  of  tumors,  in 
disseminate  sclerosis,  in  old  syphilitic  cases.  It  is  present  in  paresis  in  a 
diffuse  form  in  association  with  meningitis  (meningo-encephalitis) . 

In  children  chronic  encephalitis  presents  special  features  which  are  so 
constant  and  characteristic  that  they  form  an  important  chapter  in 
Nosology  and  known  under  the  name  of  Encephalopathies. 

Chronic  inflammation  of  the  brain  tissue  may  be  congenital  or  acquired 
in  childhood.  There  are  several  forms  of  infantile  affections  which  are 
due  to  a  chronic  encephalitis  in  intrauterine  lif e  or  developed  early  in  life. 
The  existence  of  this  condition,  although  well  known  before  Charcot,  was 
ignored  until  Striimpell  called  attention  to  his  polio-encephalitis  of  infancy. 

It  has  been  observed  that  in  the  foetus  in  utero  or  during  labor  or 
shortly  after  birth  traumatic  encephalitis,  hemorrhagic  foci,  softening  may 
occur  and  they  may  lead  to  cerebral  sclerosis,  to  diffuse  meningitis,  to 
porencephaly,  to  atrophic  condition  of  one  or  more  lobes.  In  1834 
Lallemand  had  shown  that  various  forms  of  malformation  of  the  brain 
(agenesis)  were  the  result  of  an  encephalitis.  This  view  was  corroborated 
by  Charcot  and  especially  by  Bourneville,  who  perhaps  more  than  any 
one  else  had  the  opportunity  to  deal  with  idiots  and  defective  children  of 
all  kinds  and  verify  the  clinically  observed  symptoms  on  the  post-mortem 
tables.  There  are  two  special  forms  which  will  be  considered  here; 
viz.  spastic  infantile  hemiplegia  and  Little's  disease  (Diplegia,  Paraplegia) . 


INFLAMMATION   OF   THE  BRAIN  113 

Etiology. — Traumata  of  the  pregnant  uterus,  trauma  of  the  foetus 
during  a  protracted  labor  with  or  without  application  of  forceps  not 
infrequently  lead  to  meningeal  hemorrhages  over  the  motor  area  and 
eventually  to  changes  in  the  cortex.  Experience  teaches  that  the  abnor- 
mal conditions  which  cause  long  and  difficult  labor  are  more  apt  to 
produce  birth  paralysis  than  a  proper  application  of  instruments.  Little 
lays  special  emphasis  on  dystocia  and  especially  on  premature  birth, 
which  is  a  proof  that  the  condition  may  occur  before  birth.  Strtimpell 
observed  that  infectious  diseases  are  the  special  cause  of  encephalitis  in 
extra-uterine  life.  The  hemiplegic  form  is  more  frequently  acquired  than 
congenital.  It  occurs  usually  in  the  course  of  measles,  scarlet  fever, 
typhoid,  diphtheria,  whooping  cough,  mumps  and  finally,  although  very 
rarely,  in  cases  of  endocarditis.  Whether  it  is  due  to  the  infectious 
element  itself  or  to  an  embolism  or  venous  thrombosis  (Gowers)  or  to  a 
hemorrhagic  influence  in  the  motor  area,  it  is  difficult  to  say.  As  to 
predisposing  causes,  syphilis  (Fournier)  and  alcoholism  may  be  mentioned. 
Bourneville's  researches  show  that  parental  alcoholism  showed  its  effect 
in  41  per  cent,  of  cases.  Finally,  chronic  metallic  intoxications,  as 
lead,  mercury,  phosphorus,  have  also  been  mentioned  as  causes  of  infantile 
encephalopathies.  The  present  tendency  is  to  find  the  predominant 
etiologic  role  in  infections  which  have  its  effect  not  only  on  the  foetus 
during  pregnancy  but  also  on  the  character  of  the  labor.  In  case  of 
little's  disease  which  were  apparently  due  to  abnormal  birth,  lesions 
have  been  found  which  existed  prior  to  birth.  Syphilis  for  example  has 
been  observed  with  premature  labor  or  in  still-born  children. 

Meningeal  hemorrhages  during  birth  deserve  special  mention.  Not 
only  traumatism  during  labor,  but  also  non-traumatic  cases  are  met  with 
in  which  spontaneous  hemorrhages  may  occur  in  the  foetus.  The  latter 
are  due  to  a  special  fragility  of  the  blood-vessels,  especially  the  veins, 
fragility  which  is  due  to  hereditary  syphilis,  alcohol,  lead  intoxication  or 
to  debility  from  any  cause.  The  largest  of  the  sub- arachnoid  veins,  which 
are  usually  ruptured,  are  located  along  the  anterior  and  posterior  borders 
of  the  parietal  bone,  which  correspond  to  the  lambdoid  and  coronary 
sutures.  The  coronary  suture  corresponds  to  Rolandic  area  and  conse- 
quently a  hemorrhage  at  this  level  produces  motor  symptoms,  which  is 
ordinarily  the  case.  They  are  hemiplegia,  monoplegia,  tremor,  convul- 
sions. If  convulsions  do  not  disappear,  eventually  the  following  mani- 
festations will  be  observed:  arrested  mental  development,  Little's  disease, 
disturbance  of  vision,  ocular  palsies,  deafness,  various  malformations  in 
the  limbs,  disturbances  of  speech.  It  is  therefore  evident  that  the  rec- 
ognition of  meningeal  hemorrhages  in  newly  born  infants  is  a  matter  of 


114  INELAMMATION   OF   THE  BRAIN 

great  importance.  As  soon  as  they  are  recognized ,  operative  procedures 
become  urgent  (see  Treatment). 

Pathology. — Various  conditions  may  be  found  in  the  brain.  Foei  of 
softening  or  hemorrhage  do  not  differ  to  any  marked  extent  from  those  of 
adult  brains;  the  only  pecularity  lies  in  the  retraction  of  brain  tissue  around 
the  old  morbid  foci.  Evidences  of  chronic  meningitis  are  frequently  seen 
with  the  naked  eye.  The  retracted  nervous  tissue  forms  a  depression, 
is  yellow  in  color  (yellow  plaques)  and  is  particularly  noticeable  in  the 
motor  area.  The  thickened  dura  is  adherent  to  the  cranium,  the  thickened 
pia  is  adherent  to  the  convolutions,  from  which  it  cannot  be  detached. 
Sometimes  small  cysts  are  seen;  they  are  the  result  of  old  hemorrhagic 
foci.  The  cortex  itself  undergoes  changes:  cells  and  nerve  fibers  are  in  a 
state  of  degeneration  (see  pathology  in  Apoplexy).  Briefly  speaking, 
the  condition  is  one  of  a  meningo-encpehalitis. 

Very  probably  in  many  cases  of  congenital  paralysis  intra-cranial 
hemorrhage  is  the  chief  cause.  H.  Cushing  (Amer.  J.  Med.  Sc,  1905) 
operated  on  a  series  of  such  cases  immediately  after  birth  and  found  the 
above  condition;  he  removed  the  clots  and  some  of  the  children  continued 
to  develop  normally. 

Old  lesions  may  lead  to  a  cerebral  peculiarity  named  "porencephaly." 
It  is  characterized  by  a  cavity  which  opens  on  the  surface  of  the  brain. 
The  depth  of  the  cavity  is  various :  it  may  involve  only  a  small  portion  of 
the  brain  tissue  or  communicate  with  the  lateral  ventricles.  These  cavi- 
ties are  usually  bilateral  and  they  are  found  mostly  in  the  motor  area, 
third  frontal  and  first  temporal  convolutions.  It  is  remarkable  that  they 
have  always  been  observed  in  areas  having  a  well  defined  arterial  supply. 
This  led  to  the  view  that  a  vascular  influence  is  the  cause  of  porencephaly: 
a  hemorrhage  or  softening  will  cause  a  destruction  with  a  subsequent 
cavity;  in  this  case  the  cavity  does  not  reach  the  ventricles.  The  com- 
municating cavities  may  be  also  the  result  of  a  congenital  deficiency  of 
the  blood  supply  in  a  certain  area  of  the  brain.  An  old  lesion  in  the  form 
of  an  inflammation,  limited  to  the  walls  of  the  lateral  ventricles,  involving 
the  ependyma  (ependymitis)  leads  to  another  peculiarity,  viz.  internal 
hydrocephalus.  It  is  characterized  by  a  considerable  accumulation  of 
fluid  in  the  ventricles.  The  cerebral  tissue,  which  is  in  immediate  contact 
with  the  walls  of  the  ventricle,  is  destroyed,  and  the  convolutions  being 
under  continuous  pressure,  become  atrophied.  Atrophy  of  brain  tissue 
was  also  observed  in  a  number  of  cases  as  a  result  of  a  primary  sclerosis 
of  the  brain.  This  condition  usually  affects  one  hemisphere.  The 
convolutions  are  found  thin,  retracted,  indurated,  lighter  in  color  than 
normal  cerebral  tissue.     If  only  one  hemisphere  is  affected,  the  difference 


INFLAMMATION    OF    THE   BRAIN  115 

between  the  two  halves  of  the  brain  is  striking.  It  may  happen  that  only 
one  or  several  lobes  of  the  same  hemisphere  are  affected  (microgyria). 
The  microsopical  lesion  consists  of  a  pronounced  proliferation  of  neuroglia, 
also  of  thickening  of  the  walls  of  the  capillaries  and  multiplication  of  the 
latter.  The  retraction  of  the  newly  formed  fibrous  tissue  leads  to  a 
dilatation  of  the  perivascular  spaces.  These  changes  are  present  in  the 
gray  as  well  as  in  the  white  matter,  more  marked  in  the  former  than  in  the 
latter:  the  cells  gradually  change  their  form  and  finally  disappear.  In- 
stead of  atrophic  sclerosis  the  brain  presents  sometimes  a  hypertrophy  of 
some  of  its  portions.  As  to  the  cause  of  cerebral  sclerosis,  it  is  generally 
admitted  after  Virchow  to  be  due  to  a  congenital  chronic  encephalitis,  in 
which  all  the  elements  of  the  cerebral  tissue  are  affected. 

The  above  described  morbid  conditions  developed  in  the  foetus  or 
early  in  life  present  a  special  feature  which  makes  them  so  different 
from  similar  states  in  adults  that  special  mention  should  be  made.  In 
the  chapter  on  apoplexy  we  saw  that  a  lesion  in  the  brain  inevitably 
leads  to  secondary  descending  degenerations  in  the  pyramidal  tract.  In 
the  foetus  or  in  early  infancy  the  pyramidal  tract  is  not  yet  completely 
developed,  as  the  myelin  covering  the  axis-cylinders  begins  to  appear 
only  during  the  first  months  after  birth.  It  stands  to  reason  that  a  lesion 
in  the  brain  at  those  periods  of  life  leads  to  an  arrest  of  development  and 
atrophy  of  the  portions  of  the  nervous  tissue  beneath  the  initial  lesion. 
Atrophy  and  diminution  in  size  of  nervous  tissue  (brain  and  cord)  are 
therefore  characteristic  of  the  lesions  in  question. 

Symptoms.  A.  Infantile  Spastic  Hemiplegia. — The  symptoms  of 
spastic  paralysis  may  be  noticed  at  birth  or  only  when  the  child  makes 
the  first  attempt  to  walk.  In  some  cases  the  onset  of  paralysis  is  pre- 
ceded by  a  group  of  acute  symptoms:  fever,  restlessness,  vomiting. 
Convulsions  appear  early  if  not  first;  they  are  epileptiform  in  character 
and  confined  at  first  to  the  side  which  is  to  become  paralyzed.  The 
spasms  soon  become  generalized  and  increase  in  frequency.  During 
these  epileptic  attacks,  which  usually  last  about  forty-eight  hours,  the 
hemiplegia  suddenly  sets  in.  It  is  at  first  flaccid,  affects  leg,  arm  and 
face;  the  distal  ends  of  the  extremities  are  the  most  affected  and  the 
upper  extremity  more  than  the  lower.  At  the  end  of  ten  or  fifteen  days 
spasticity  gradually  develops,  all  the  reflexes  become  exaggerated,  ankle- 
clonus,  Babinski's  sign  and  paradoxical  reflex  are  easily  elicted  (see 
Apoplexy) .  The  hemiplegia  is  established  and  remains  permanent  (Fig. 
67).  While  in  a  general  way  it  is  similar  to  hemiplegia  of  adults,  it 
nevertheless  presents  these  peculiarities  that  the  contractures  with  the 
subsequent  deformities   are  very  marked:  the  flexion  of  the  hand  and 


n6 


INFLAMMATION    OF    THE   BRAIN 


fingers,  the  varus  equinus  are  pronounced  to  a  great  extent.  The  striking 
and  distinguishing  feature  of  infantile  spastic  hemiplegia  is  the  atrophy 
of  the  entire  affected  side:  skin,  fat,  connective  tissue,  musculature 
and  bone  participate  in  the  atrophic  process;  not  only  the  limbs,  but 
also  the  face  and  thorax  show  diminution  in  size.  Deformity  of  the 
skeleton,  particularly  scoliosis,  is  observed.    The  atrophy  is  more  marked 

in  the  upper  extremity  than  in  the 
lower.  Vasomotor  disturbances,  as 
well  as  sensory,  are  present:  the  skin 
is  cold,  the  sensations  are  diminished. 
Astereognosis  (see  page  69)  is  always 
present  in  view  of  the  arrested  develop- 
ment of  the  hand  at  an  early  age. 

The  paralyzed  limbs  are  not  in- 
frequently affected  with  athetosis  or 
choreic  movements  (hemiathetosis  and 
hemichorea).  In  these  cases  the  spas- 
ticity is  not  pronounced.  Occasion- 
ally both  varieties  of  muscular  move- 
ments are  combined.  Sometimes  the 
athetosis  is  bilateral.  In  some  cases 
there  is  an  intention  tremor,  such  as 
is  seen  in  disseminated  sclerosis. 
Athetosis,  chorea,  tremor  may  vary: 
they  may  change  in  intensity  at  a 
certain  time  of  the  day  or  they  may 
substitute  each  other.  They  totally 
disappear  during  sleep. 

Among  other  complications  of  in- 
fantile spastic  hemiplegia  may  be 
mentioned  Aphasia,  but  the  latter  is 
an  exceptional  occurrence.  More 
frequently  there  is  a  delayed  development  of  speech  faculty,  which  begins 
to  develop  only  at  the  age  of  four  or  five  or  even  later,  or  after  the  speech 
has  developed  it  remains  deficient  for  a  long  time  and  even  permanently. 
True  aphasia  is  not  present.  The  intellectual  faculties  are  quite  frequently 
affected  in  hemiplegia,  but  more  frequently  when  the  frontal  lobe  is 
congenitally  diseased  than  the  Rolandic  area.  In  view  of  the  tender 
age  of j  the  child  there  is  a  retardation  of  mental  development  in  almost 
every  case.     Imbecility  and  idiocy  are  observed  in  some  cases. 

Children  born  hemiplegic  sometimes  present  defects  in  the  sphere 


Fig.  67. — R.  Infantile  Hemiplegia. 


INFLAMMATION    OF    THE    BRAIN 


117 


of  the  special  senses,  as  congenital  strabismus,  hemianopsia,  nystagmus, 
deafness. 

Epilepsy  is  quite  a  frequent  complication.  It  may  exist  from  early 
infancy  or  make  its  appearance  later,  especially  around  puberty.  The 
convulsions  may  be  confined  to  the  paralyzed  side  or  be  generalized.  In 
the  latter  case  there  is  usually  loss  of  consciousness  with  biting  of  the 
tongue,  frothing  at  the  mouth  and  other  symptoms  characteristic  of 
essential  epilepsy  (see  Epilepsy).  In  many  cases  the  epilepsy  has  a  ten- 
dency to  decrease  with  age,  but  in  others  remains  permanently.  Petit 
mal  attacks  occur  usually  in  cases  in  which  the  paralysis  is  slight. 

B.  Spastic  Diplegia.    Little's  Disease. 

In  the  chapter  on  Pathology  one  could  see  the  various  morbid  con- 
genital conditions  which  are  apt  to  create  the  paralytic  states  observed 
during  life.  When  the  seat  of  the  lesion,  instead  of  being  unilateral, 
is  symmetrically  distributed  to  the  motor  areas  of  both  hemispheres, 
the  clinical  manifestation  will  be  a  double  hemiplegia  or  diplegia.  When 
the  lesion  affects  only  both  paracentral  lobules  a  double  paralysis  of 
both  lower  extremities  will  be  observed  (paraplegia).  These  are  the 
two  clinical  varieties  usually  met  with  in  practice.  On  the  other  hand, 
if  during  a  protracted  and  difficult  labor  or  after  birth  hemorrhages 
should  occur  in  the  brain  or  in  the  spinal  cord,  a  double  hemiplegia 
(diplegia)  or  paraplegia  (palsy  of  both  lower  extremities)  may  equally 
occur  (Figs.  68  and  69).  Spastic  diplegia  or  paraplegia  may  therefore 
be  observed  in  two  different  conditions:  congenital  and  acquired.  One 
of  the  earliest  writers  who  called  attention  to  the  first  variety  was  Little, 
who  in  1862  gave  the  best  description.  He  pointed  out  as  the  most 
important  etiological  moment  of  the  affection  premature  labor.  He 
observed  that  some  children  born  prematurely  presented  early  symptoms 
of  spastic  diplegia.  It  is  therefore  natural  to  suppose  that  in  those 
cases  a  deficient  development  of  the  pyramidal  tract  (agenesis)  was  the 
direct  cause  of  the  malady.  It  is  reasonable  to  presume  in  the  light  of 
our  present  knowledge  especially  with  reference  to  Wasserman  reaction 
that  premature  labor  and  Little's  diseases  are  the  consequence  of  the 
same  cause,  viz.  hereditary  syphilis.  In  a  very  large  number  of  cases 
of  diplegic  Wasserman  test  has  been  found  to  be  positive.  At  present 
the  writers  are  divided:  some  describe  under  the  name  of  Little's  disease 
both  the  congenital  and  the  acquired  forms,  some  make  a  sharp  distinc- 
tion and  ccnsider  three  characteristic  features  necessary  for  Little's  dis- 
ease, viz.  (1)  the  affection  must  be  congenital,  (2)  of  cerebral  origin,  and 
(3)  due  to  agenesis  of  the  pyramidal  tract.  The  pyramidal  fibers  become 
covered  with  myelin  only  during  the  first  few  months  after  normal  birth. 


n8 


INFLAMMATION    OF    THE   BRAIN 


According  to  Van  Gehuchten,  at  seven  months  the  pyramidal  fibers  are 
absent  in  the  spinal  cord  and  can  be  traced  only  in  the  brain  and  medulla. 
It  stands  to  reason  that  there  is  no  continuity  between  the  cortex,  which 
sends  out  stimulation,  and  the  peripheral  nerves,  or  else  the  stimulation 
sent  out  from  the  cortex  to  the  spinal  centers  is  not  properly  transmitted 
for  want  of  normal  pyramidal  fibers.  The  rigidity  of  the  muscles  in 
diplegia  can  therefore  be  explained  either  by  increase  of  the  muscular 


Fig.  68. — Diplegia,  Little's 
Disease. 


Fig.  69. — Paraplegia,  Little's  Disease  (Idiot). 


tonus  or  by  transmission  of  a  continuous  morbid  stimulation  through 
imperfectly  developed  pyramidal  fibers. 

Fournier  had  long  ago  called  attention  to  hereditary  syphilis  as  an  etio- 
logical factor  in  Little's  disease.  In  a  large  number  of  cases  the  disease 
is  present  in  individuals  whose  parents  were  distinctly  syphilitic.  Not 
infrequently  diplegic  children  present  certain  stigmata  of  syphilis,  such 
as  hydrocephalus,  internal  strabismus,  etc.     In  some  cases  at  autopsy 


INFLAMMATION    OF    THE  BRAIN  119 

of  diplegics  lesions  of  syphilitic  nature  had  been  found:  sclerotic  and 
gummatous  formations,  meningeal  conditions,  cerebral  endarteritis, 
meningo-myelitis.  Finally,  Wasserman  reaction  has  been  frequently 
found  positive  in  diplegics.  On  the  other  hand,  a  negative  Wasserman 
does  not  exclude  syphilis.  The  fact  that  diplegia  is  observed  frequently 
in  association  with  premature  birth  is  also  suggestive  of  hereditary 
syphilis. 

Symptoms. — The  most  frequent  form  is  the  paraplegic.  The  gait 
and  station  are  characteristic:  the  patient's  trunk  is  bent  forward,  he 
looks  at  his  feet,  which  are  in  a  state  of  varo-equinus.  When  standing 
the  thighs  are  in  close  contact  with  each  other,  while  the  legs  are  separated. 
In  walking  the  legs  have  a  tendency  to  cross  each  other,  the  feet  make 
a  hemi-circle  movement,  and  scrape  the  floor  at  each  attempt  to  advance 
and  the  trunk  turns  laterally.  When  seated,  the  legs  do  not  touch  the 
floor;  they  are  extended.  The  rigidity  is  so  great  that  flexion  of  the  joints 
is  impossible. 

In  the  diplegic  form  the  upper  extremities  are  also  affected,  but 
to  a  milder  degree:  adduction  of  the  arms,  flexion  and  pronation  of 
the  forearms  are  the  principal  signs.  The  face  not  infrequently  partici- 
pates in  the  general  rigidity.  When  the  patient  speaks,  laughs  or  mas- 
ticates, there  is  a  very  marked  contortion  of  the  muscles  of  the  face; 
the  speech  is  therefore  indistinct.  Should  the  muscles  of  deglutition, 
phonation  or  respiration  be  affected,  corresponding  symptoms  will  be 
present.  They  constitute  the  congenital  pseudo-bulbar  paralysis;  succion, 
deglutition  are  disturbed;  later  the  speech  shows  slowness  and  words 
are  pronounced  in  an  explosive  manner.  The  contracture  of  the  ex- 
tremities may  not  be  evenly  distributed  and  its  intensity  may  vary  in 
different  limbs. 

The  condition  of  the  reflexes  is  the  same  as  in  the  hemiplegic  form, 
viz.  exaggerated  patellar  or  other  tendon  reflexes,  Babinski  and  paradox- 
ical flexor  signs;  ankle-clonus  is  rare.  Athetosis  is  not  infrequently 
observed  in  both  hands.  Choreic  movements  are  less  frequent.  The 
mental  condition  in  Little's  disease  deserves  special  mention.  While 
the  children  thus  affected  present  some  delay  in  development  of  their 
mental  faculties,  they  nevertheless  do  not  show  marked  impairment  of 
intelligence,  and  in  some  cases,  with  proper  training,  their  intellect  may 
reach  a  normal  degree  of  development.  There  are  also  cases  of  infantile 
encephalopathies  of  the  type  of  diplegia  in  which  idiocy  and  imbecility 
are  observed.  In  these  cases  epilepsy  is  very  frequently  associated. 
Diplegics  not  infrequently  present  a  peculiar  excitability  in  regard  to 
noises,  what   Oppenheim   called   "abnorme    Schreckhaftigkeit."     When 


120  INFLAMMATION    OF    THE   BRAIN 

they  hear  an  object  fall  on  the  floor  or  a  noise  of  any  sort,  they  are  seized 
with  a  trembling  which  may  last  a  long  time.  The  condition  is  an 
acustico-motor  hyperesthesia. 

Prognosis,  (a)  In  Infantile  Hemiplegia—  Generally  speaking,  it  is 
unfavorable.  The  contracture  and  the  atrophy,  viz.  the  arrested  devel- 
opment of  the  tissues  of  the  palsied  limbs,  render  the  infirmity  permanent. 
Improvement  of  the  paralytic  symptoms  may  follow,  especially  during  the 
first  year,  but  it  is  usually  slight.  Epilepsy  is  a  very  grave  complication, 
as  it  prevents  any  possible  amelioration  of  symptoms  and  retards  the 
development  of  the  mental  faculties.  Epilepsy  at  the  onset  of  the  affec- 
tion may  cause  rapid  death.  According  to  Bourneville  the  attacks  of 
epilepsy  become  rarer  as  the  child  grows  and  often  disappear  at  the  age  of 
thirty.  The  development  of  mental  faculties  is  usually  delayed,  but 
with  a  proper  training  good  results  may  be  obtained. 

(b)  In  Diplegia.- — The  prognosis  in  this  affection  is  less  unfavorable 
than  in  the  hemiplegic  form.  There  is  a  natural  tendency  toward 
improvement,  but  recovery  can  never  be  expected. 

Diagnosis. — The  spasticity  of  the  paralyzed  extremities,  marked 
contractures,  athetosis  or  chorea  with  epileptiform  convulsions,  the 
period  of  life  at  which  these  symptoms  make  their  appearance  are  usually 
sufficient  facts  for  making  a  diagnosis.  In  some  cases,  however,  difficul- 
ties arise.  When  the  infantile  spastic  hemiplegia  presents  an  acute 
onset,  it  may  simulate  tubercular  meningitis.  In  such  cases  the  diagnosis 
cannot  be  made  at  the  beginning:  the  course  of  the  affection  alone  will 
enable  one  to  make  a  correct  diagnosis. 

Infantile  spinal  palsy  also  has  an  acute  onset,  but  the  flaccidity 
of  the  palsy  alone  besides  the  diminution  or  less  of  reflexes  is  sufficient  to 
determine  the  disease. 

Obstetrical  palsy,  limited  usually  to  one  upper  extremity,  can  be 
recognized  by  its  flaccidity. 

Tumor  of  the  brain  will  be  recognized  from  the  slow  onset,  from  the 
history  of  headache  and  vertigo  and  especially  from  the  condition  of 
the  eye  grounds  (optic  neuritis  or  atrophy,  or  choked  disc). 

Little's  disease,  especially  the  diplegic  form,  is  so  characteristic  (see 
above)  that  it  cannot  be  easily  confounded  with  other  affections.  The 
paraplegic  variety  will  be  differentiated  from  paraplegia  caused  by  mye- 
litis mainly  by  the  absence  of  disturbance  of  the  sphincters  of  bladder 
and  rectum,  also  by  the  history  of  the  case. 

Treatment. — In  the  chapter  on  etiology,  among  various  causes'~of 
spastic  paralysis,  dystocia  was  considered  as  a  possible  factor.  It  is 
therefore  evident  that  prompt  obstetrical  intervention   during  difficult 


INFLAMMATION    OF    THE  BRAIN  121 

labor  is  an  important  preventive  measure.  When  the  child  is  born  nor- 
mally and  easily  and  still  presents  spastic  paralysis,  the  condition  is  con- 
genital. When  the  hemiplegia  preceded  by  the  group  of  acute  symptoms 
(see  Symptomatology)  appears  some  time  after  birth,  the  treatment  will 
not  be  practically  different  from  an  apoplectic  insult  in  an  adult.  It  is 
the  treatment  of  deformities  and  infirmities  that  we  are  requested  to 
remedy.  Internal  drugs  are  of  no  value  except  for  epileptiform  convul- 
sions, in  which  case  bromides  are  indicated.  However  the  relation  of 
diplegia  to  hereditary  syphilis  (page  349)  is  a  strong  indication  for  the 
use  of  antispecific  medications,  viz.  mercury,  iodids  and  especially  sal- 
varsan.  Mechanical  means  and  surgical  intervention  is  the  only  possible 
treatment.  It  is  well  understood  that  conditions  like  porencephaly, 
internal  hydrocephalus,  sclerosis  and  atrophy  of  the  brain  can  never  be 
benefited  by  operative  procedures.  When  the  paralytic  or  epileptic  symp- 
toms are  due  to  a  vascular  lesion  or  to  a  cyst,  will  an  operation  give  a 
permanent  recovery?  Is  it  justifiable  to  perform  even  an  exploratory 
operation?  Statistics  carefully  collected  show  that  some  improvement 
was  observed  in  a  certain  group  of  cases,  but  as  the  cases  were  not  kept 
under  observation  a  sufficiently  long  time,  it  is  impossible  to  draw  positive 
conclusions.  Moreover  in  a  small  group  of  cases  the  slight  improvement 
was  only  temporary  and  a  number  of  fatal  results  were  also  reported. 
However,  more  recent  investigations,  especially  those  of  Cushing  in  1905, 
show  that  in  many  cases  of  congenital  paralysis  hemorrhage  within  the 
cranium  at  birth  is  the  main  etiological  factor.  Prompt  and  early  surg- 
ical intervention  for  removal  of  blood-clots  gave  him  in  some  cases  very 
satisfactory  results.  He  opens  the  skull  at  the  level  of  the  parietal  region, 
makes  a  large  opening  in  the  dura,  and  washes  out  the  blood.  A 
similar  operation  is  done  on  the  other  side  of  the  brain.  In  view  of  the 
great  gravity  of  such  operations  in  newly  born  infants  Gilles  devised  a 
method  which  apparently  gives  promising  results.  In  cases  in  which  a 
meningeal  hemorrhage  is  suspected  a  puncture  of  the  anterior  fontanelle 
should  be  made.  It  decreases  intra-cranial  pressure  and  reduces  the  dan- 
ger of  infection  caused  by  absorption  of  toxic  products  from  the  blood 
clot. 

This  is  in  Gilles'  opinion  a  very  good  preparatory  treatment.  When 
the  brain  becomes  more  developed  and  the  child  is  stronger,  a  radical  opera- 
tion may  be  undertaken,  such  as  Cushing's.  Gilles'  rule  is  at  first  to  per- 
form a  lumbar  puncture  and  when  the  latter  gives  no  relief ,  puncture  the 
anterior  fontanelle  (see  also  page  166). 

Surgery  directed  toward  the  deformities  and  contractures  is  apt  to 
give  favorable  results.     Tenotomy  and  myotomy  followed  by  applica- 


122  INFLAMMATION    OF   THE  BRAIN 

tion  of  plaster  casts  to  the  limbs  put  in  corrected  positions  has  given 
favorable  results  in  certain  cases.  Various  orthopedic  appliances,  pas- 
sive manipulations  of  the  limbs,  mobilization  of  the  joints  and  properly- 
regulated  gymnastics  are  highly  commendable  for  counteracting  the 
rigidity  of  the  muscles  and  of  their  tendons.  Massage  is  a  great  adju- 
vant in  this  treatment;  it  should  never  be  overlooked,  as  very  good  re- 
sults are  sometimes  obtained.  It  is  a  good  plan  to  administer  frequently 
warm  baths  which  will  f  acilitate  the  reduction  of  the  muscular  spasticity. 
Electricity  is  not  advisable  as  it  is  likely  to  increase  the  muscular  rigidity. 

Recently  Forster  (Beitr.  z.  Klin.  Chir.,  1909)  has  suggested  to  resect  the 
posterior  spinal  nerve  rcots  for  relief  of  spasticity.  He  based  this  oper- 
ation upon  the  following  physiological  considerations.  Spastic  paraly- 
sis is  due  to  a  diseased  condition  of  the  corticospinal  tract,  especially  the 
pyramidal  tract.  The  latter  carries  two  kinds  of  fibers:  those  bearing  the 
motor  impulse,  a  disease  of  which  causes  the  paralysis,  also  those  contain- 
ing inhibitory  fibers  whose  function  is  to  check  the  sensory  stimuli  received 
from  the  sensory  roots  of  the  spinal  cord.  The  spasticity  is  therefore  a 
reflex  act.  As  a  result  of  the  damage  to  the  inhibitory  fibers,  the  con- 
tinuous sensory  impulses  act  unrestrained,  hence  the  spasticity.  When 
in  such  cases  the  posterior  roots  are  cut,  the  sensory  afflux  is  removed  and 
the  spasticity  becomes  diminished  or  disappears  entirely.  A  large  num- 
ber of  operations  have  since  been  performed  and  in  the  majority  of  cases 
satisfactory  results  have  been  obtained.  Spastic  diplegia  and  hemi- 
plegia can  be  relieved  considerably  by  Forster's  operation.  For  the 
upper  extremity  the  last  four  cervical  roots  and  for  the  lower  extremity  the 
last  three  or  four  lumbar,  also  the  first  and  second  sacral  roots  should  be  re- 
moved on  both  sides.  The  after-treatment  is  very  important.  Forster 
insists  upon  careful  exercises  of  the  limbs,  which  must  continue  for  years 
after  the  operation.  Immediately  following  the  operation  the  limbs 
should  be  placed  in  removable  plaster  splints  in  corrected  positions. 
The  splints  will  be  kept  on  for  a  very  long  time  and  removed  off  and  on 
only  for  the  exercises,  which  must  be  carried  out  several  times  a  day. 

Recently,  Schwab  and  Allison  (Trans.  Amer.  Neur.  Ass'n.,  1910), 
devised  a  method  of  treatment  of  spasticity  and  athetosis  which  they 
called,  "muscle  group  isolation." 

It  consists  of  isolating  muscles  or  groups  of  muscles  which  are  at 
fault  in  the  production  of  contracture,  deformity  or  athetosis.  It  is  done 
by  cutting  off  from  the  central  nervous  system  the  connection  along 
which  the  abnormal  impulse  is  transmitted.  This  is  accomplished  by 
isolating  the  nerve  innervating  the  affected  muscles  and  injecting  it 
with  alcohol.     A  paralysis  of  these  muscles  will  thus  be  induced  with- 


INFLAMMATION   OF    THE  BRAIN  1 23 

out  interfering  with  the  antagonistic  muscles.  The  subsequent  treat- 
ment will  consist  of  massage  and  physiological  exercises.  In  Little's  dis- 
ease for  example  the  obturator  nerve  which  supplies  the  adductor  muscles 
of  the  thigh  is  to  be  injected  with  alcohol.  This  method  is  also  advis- 
able in  deformities  of  anterior  poliomyelitis. 

Another  method  for  "muscle  isolation"  in  treatment  of  spastic  paraly- 
sis has  been  devised  by  Stoffel  (Munch,  med.  Wchn.,  191 1  and  1912).  His 
operation  aims  to  weaken  the  contracted  muscle  by  depriving  it  of  a  cer- 
tain part  of  its  innervation,  while  at  the  same  time  the  antagonist  muscle 
is  strengthened  by  massage  and  exercises.  When  the  nerve  is  exposed 
for  3  or  4  cm.,  its  different  fibers  are  touched  with  a  needle  electrode 
carrying  a  current  so  weak  that  the  innervated  muscle  barely  twitches. 
It  is  thus  possible  to  single  out  the  nerve  fibers  involved.  Thus  instructed 
the  tract  innervating  the  muscle  involved  in  the  contracture  is  worked 
loose  for  a  distance  of  5  or  7  cm.  The  nerve  bundle  is  then  severed 
in  cases  of  severe  contracture,  or  only  part  of  the  fibers.  The  after- 
treatment  is  very  important.  When  the  operation  is  correctly  performed 
and  suitable  after-care  with  massage  and  exercise  is  given,  there  will  be 
a  certainty  of  success  in  correcting  spastic  contracture  of  any  form  and 
of  any  duration.  Stoffel  gives  a  final  review  of  his  many  cases,  almost 
all  ending  successfully,  in  Therap.  Monatshefte,  Dec,  19 12. 


CHAPTER     VI 

JACKSONIAN  OR  FOCAL  EPILEPSY 

The  existence  of  a  special  form  of  epilepsy  and  different  from  the 
ordinary  generalized  epilepsy  was  observed  first  in  hemiplegics  on  the 
paralyzed  side  of  the  body  by  Bravias,  but  H.  Jackson  (1869)  deserves 
the  credit  of  demonstrating  the  relationship  between  a  unilateral  epilepsy 
and  a  cortical  lesion  of  the  brain. 

Pathology. — It  is  definitely  established  that  Jacksonian  or  focal  or 
partial  epilepsy  is  caused  by  an  irritation  of  the  cortex  of  the  motorarea 
of  the  opposite  side.  The  focus  of  irritation  may  be  situated  in  the  kull 
(pressure  of  a  fragment  in  traumata),  in  the  meninges  (pachymeningitis, 
syphilitic  meningo- encephalitis),  in  the  cortex  itself  and  in  the  ub- 
cortical  tissue  (tumors,  etc.).  Whether  the  irritation  is  from  without 
or  from  within,  the  excitation  of  the  motor  cells  of  the  Rolandic  area  is 
indispensable  to  produce  a  focal  epilepsy.  Sometimes  the  irritative 
lesion  is  found  outside  the  motor  area.  In  such  cases  the  irritation  of 
the  cortical  cells  of  the  motor  region  is  set  up  secondarily.  Irritation 
of  the  cells  produces  tonic  followed  by  clonic  convulsions,  while  irritation 
of  the  subjacent  nerve-fibers  alone  produces  only  tonic  convulsions. 

Symptoms. — An  attack  of  Jacksonian  epilepsy  consists  of  tonic  and 
mainly  clonic  convulsions  which  begin,  mostly  without  loss  of  conscious- 
ness, in  a  very  limited  group  of  muscles  on  one  side  and  which  rapidly 
spread  to  a  half  of  the  body  and  sometimes  to  the  entire  body.  Ac- 
cording to  the  point  of  departure  of  the  convulsions  three  types  can  be 
considered,  viz.  facial,  brachial  and  crural.  In  the  first  type  the  spasm 
affects  the  face  and  neck.  Either  one  angle  of  the  mouth  is  drawn  up 
or  one  eye  is  rolled  up;  the  head  is  turned  toward  the  same  side.  The 
spasmodic  contractions  become  precipitated  and  soon  invade  the  muscles 
of  the  neck  and  of  the  arm.  The  forearm  rapidly  becomes  pronated, 
the  hand  closes.  Immediately  the  lower  extremity  becomes  extended 
and  rigid.  The  clonic  contractions  follow  instantly.  In  the  second 
type,  which  is  the  most  common,  the  initial  symptom  appears  in  the 
thumb,  which  becomes  flexed  and  the  other  four  fingers  follow.  The 
hand  is  pronated,  a  rigid  flexion  of  all  the  segments  of  the  limb  sets  in 
and  the  convulsive  movements  begin  immediately.  The  face  and  the 
lower  extremity  follow. 

In  the  third  type  the  initial  symptom  appears  in  the  great  toe.     Un- 

124 


JACKSONIAN    OR   FOCAL   EPILEPSY  1 25 

like  the  upper  extremity,  here  tonic  contractions  are  rare,  clonic  move- 
ments appear  usually  from  the  beginning.  The  upper  extremity  and 
face  become  invaded  subsequently.  The  order  of  appearance  of  the 
symptoms  as  just  described  is  not  always  observed.  The  convulsions 
may  commence  in  one  area  and  remain  confined  to  the  same,  or  else  they 
may  spread  only  to  a  portion  of  the  half  of  the  body. 

In  another  group  of  cases  the  convulsions  may  become  generalized 
and  affect  the  entire  body.  In  such  cases  the  importance  of  the  knowl- 
edge of  the  onset  of  the  convulsions  cannot  be  overestimated.  The  onset 
is  the  most  precious  localizing  sign.  Surgical  intervention  is  based 
almost  exclusively  upon  this  information.  As  to  what  portion  of  the 
cortex  causes  convulsions  of  the  face,  arm  and  leg,  see  chapter  "Cerebral 
Localizations." 

The  attacks  of  Jacksonian  epilepsy  may  be  preceded,  accompanied 
or  followed  by  a  few  symptoms  which  deserve  mention.  The  aura, 
which  is  present  in  idiopathic  epilepsy,  is  quite  frequently  met  with  in 
focal  epilepsy.  It  may  be  motor,  sensory  and  psychic.  The  motor 
aura  consists  of  an  involuntary  movement  in  some  of  the  muscles,  which 
is  then  the  signal  of  an  oncoming  attack.  The  sensory  aura  is  various. 
It  may  be  a  sharp  lightning  pain,  a  sensation  of  heat,  of  cold,  a  tingling, 
etc.  The  psychic  aura  consists  of  hallucinations  (visual  and  aural) : 
patient  sees  flashes  of  light,  fire,  red  or  green  objects,  etc.  In  some  cases 
a  state  of  confusion  precedes  an  attack.  One  of  my  patients  would  run 
bewildered  immediately  before  he  would  fall  unconscious.  As  to  un- 
consciousness, during  an  attack  it  may  be  complete,  incomplete  or  en- 
tirely absent.  In  the  latter  case  the  patient  witnesses  the  entire  cycle 
of  symptoms.  When  there  is  only  partial  loss  of  consciousness,  the 
patient  has  a  vague  knowledge  of  his  personality  and  he  is  then  apt  to 
commit  acts  for  which  he  is  not  responsible.  Pallor  of  the  face,  followed 
by  cyanosis  and  involuntary  micturition,  are  two  additional  symptoms 
observed  during  the  attacks. 

After  an  attack  is  over,  the  patient  lies  motionless  and  appears  ex- 
hausted. Gradually  he  regains  consciousness,  looks  vaguely  about  him 
and  finally  recovers;  headache  frequently  follows  an  attack.  Not  un- 
commonly is  observed  after  an  epileptic  attack  a  paralysis  of  the  limbs 
involved.  The  palsy  is  usually  transitory,  may  last  twenty-four  or 
forty-eight  hours,  although  sometimes  may  remain  permanent.  In  the 
latter  case  there  is  probably  a  certain  permanent  damage  of  the  cortical 
tissue  (hemorrhage  followed  by  softening).  The  transitory  post-epileptic 
paralysis  is  flaccid,  but  increased  knee-jerk  and  sometimes  Babinski's 
sign  will  be  observed.     The  paradoxical  reflex  has  been  observed  by  me 


126  JACKSONIAN   OR   FOCAL   EPILEPSY 

in  every  case  that  came  under  my  observation  and  disappeared  when 
the  paralysis  disappeared. 

Varieties. — Besides  the  typical  attack,  in  which  the  tonic  convulsions 
are  followed  by  clonic,  there  is  a  form  which  consists  exclusively  of  tonic 
contractions;  the  latter  then  constitutes  the  entire  attack.  In  such 
cases  the  irritative  lesion  is  in  the  nervous  fibers  subjacent  to  the  cortical 
cells.  Finally  there  is  a  sensory  variety,  in  which  the  entire  attack  con- 
sists of  a  sensory  phenomenon.  A  patient  of  the  Jefferson  Hospital 
presented  sudden  brief  attacks  of  coldness  and  numbness  in  the  entire 
left  side  of  her  body.  This  lasted  only  two  minutes,  during  which  time 
she  felt  dazed;  her  face  was  pale  and  after  the  attack  was  over  she  went 
to  sleep. 

Prognosis.- — It  depends  exclusively  upon  the  cause  of  the  cortical 
irritation.  When  the  condition  is  amenable  to  surgical  intervention, 
the  outlook  is  favorable.  It  is  also  favorable  in  syphilitic  cases.  Gener- 
ally there  is  a  tendency  to  destruction  of  the  irritated  area,  so  that  event- 
ually a  paralysis    supervenes. 

Diagnosis. — The  mode  of  onset,  viz.  the  initial  appearance  of  con- 
vulsive attacks  on  one  side  of  the  body,  is  sufficient  to  make  a  differential 
diagnosis  between  focal  and  essential  epilepsy.  Hysteria  is  an  affection 
in  which  unilateral  convulsive  movements  may  simulate  Jacksonian  epi- 
lepsy, but  the  presence  of  hysterical  stigmata  and  the  character  of  hys- 
terical muscular  contractions  will  enable  a  close  observer  to  make  a  dis- 
tinction between  the  organic  cerebral  disease  and  the  functional  nervous 
disease  (see  Hysteria).  After  the  diagnosis  is  established,  it  is  highly 
important  to  ascertain  the  cause  and  localize  the  seat  of  the  irritating 
factor.  When  an  evident  injury  to  the  cranium  had  occurred  or  when 
there  are  evidences  of  an  intracranial  neoplasm,  it  is  comparatively  easy 
to  explain  the  pathogenesis  of  the  disease.  When  convulsions  are  fol- 
lowed by  a  paralytic  or  paretic  condition  of  the  face,  arm  or  leg,  the 
diagnosis  is  easily  established.  In  a  number  of  cases  it  is  difficult,  if  not 
impossible,  to  determine  the  cause  and  indeed  no  organic  lesion  is  found 
during  operation  or  post-mortem  in  a  great  many  cases.  Syphilitic  gum- 
mata  or  localized  specific  pachymeningitis  should  be  always  thought 
of  in  obscure  cases.  Wasserman  test  will  be  of  considerable  assistance 
in  such  cases.  As  to  the  localization  of  the  lesion,  it  will  be  determined 
from  a  close  observation  of  the  attacks,  as  the  seat  of  the  convulsive 
attacks  or  the  seat  of  their  beginning  will  enable  us  to  localize  the  lesion 
in  the  cortex.  X-ray  examination  may  render  a  valuable  service  in 
such  cases.  Pain  may  sometimes  be  elicited  by  pressure  over  the  area 
corresponding  to  the  suspected  irritative  lesion. 


JACKSONIAN    OR   FOCAL   EPILEPSY 


127 


Etiology. — It  has  been  already  mentioned  that  irritation  of  the  motor 
zone  of  the  cortex,  which  is  the  chief  cause  of  Jacksonian  epilepsy,  may  be 
produced  by  fragments  of  the  skull  in  traumatism,  by  hemorrhages, 
localized  meningitis,  gummata,  tumors.  All  these  factors  act  at  first 
mechanically  upon  the  cortex  and  then  cause  an  inflammatory  condition 
of  the  latter.  But  there  are  other  conditions  in  which  focal  epilepsy  has 
been  observed.     Alcoholism    and   lead   poisoning   are   two   well   known 


CENTRAL 

SULCUS 

(.ROLANDO) 

INTER- 
PARIETAL 
SULCUS 

POSITION  OF 
PARIETAL 
EMINENCE 


EXTERNAL 
PART  OF 
PARIETO- 
OCCIPITAL 

FISSURE 


POSITION  OF 
FRONTAL  EMINENCE 


SUPERIOR 
TEMPORAL 
SULCUS 


MIDDLE 

TEMPORAL 

SULCUS 


Fig.  70. — Drawing  of  a  Cast  of  the  Head  of  an  Adult  Male.     {Morris'  Anatomy.) 
(Prepared  by   Professor   Cunningham   to    illustrate   cranio-cerebral   topography.) 


intoxications,  to  which  Jacksonian  epilepsy  can  be  attributed.  That 
uremia  may  become  complicated  by  attacks  of  Jacksonian  epilepsy  is 
also  a  well-known  fact.  On  the  other  hand  peripheral  irritation,  as,  for 
example,  injury  to  nerve  trunks,  burns,  cicatrices,  polyps,  surgical 
operations,  etc.,  have  been  the  starting  cause  of  unilateral  epilepsy.  In 
such  cases  the  pathogenesis  being  obscure,  one  is  bound  to  accept  the 
view  of  a  reflex  origin  of  Jacksonian  epilepsy. 


128  JACKSONIAN    OR   FOCAL   EPILEPSY 

Treatment. — When  an  intoxication  can  be  traced  as  a  cause  of  the 
epilepsy,  medical  treatment  is  indicated  and  it  will  consist  of  the  removal 
of  the  cause.  When  syphilis  is  ascertained  or  suspected,  mercurials  and 
iodides  also  salvarsan  may  yield  excellent  results.  In  all  other  conditions 
surgical  intervention  is  justifiable.  Operations  should  be  always  preceded 
by  a  careful  mapping  out  of  the  area  involved  (Fig.  70)  and  aided  by 
X-ray  pictures.  Also  each  operation  should  be  preceded  by  a  trial  of 
specific  treatment  until  the  point  of  intolerance.  While  in  some  cases 
operations  give  brilliant  results,  in  others  the  amelioration  of  the  condition 
is  only  transitory.  Outside  of  intoxications  and  uremia  unilateral  con- 
vulsions are  always  justifiable  of  an  exploratory  operation.  The  methods 
usually  employed  are  those  of  Kocher  or  of  Horsley. 

(1)  Kocher's  method  consists  of  trephining  with  incision  of  the  dura. 
It  has  this  advantage  that  in  cases  without  an  evident  lesion  the  high 
pressure  of  the  cerebro-spinal  fluid  is  either  diminished  or  removed. 
Experimental  and  clinical  observations  show  that  the  pressure  is  in- 
creased in  epilepsy.  Kocher  believes  that  high  pressure  of  the  cerebro- 
spinal fluid  is  the  cause  of  epilepsy.  He  removes  a  small  round  piece 
from  the  skull,  excises  the  dura,  replaces  the  bone  and  covers  it  with  the 
incised  integument. 

(2)  Horsley's method  consists  of  excising  the  so-called  "signal  center. " 
In  order  to  determine  precisely  the  seat  of  this  center,  he  stimulates 
the  supposed  center  with  a  very  weak  faradic  current.  When  a  motor 
manifestation  is  reproduced,  he  excises  the  area  even  if  there  is  no  evident 
local  lesion.  A  center  apparently  normal,  he  thinks,  may  show  histolog- 
ical lesions.  Krause  adopted  as  a  guide  the  "signal  center"  and  con- 
siders it  the  most  important  element  of  surgical  diagnosis.  He  advises 
to  remove  twenty  or  twenty-five  millimeters  of  cortical  surface  and  five  to 
eight  millimeters  in  depth.  The  paralysis  and  anesthesia  that  may  follow 
the  operation  are  only  transitory  if  the  dimensions  of  the  excised  cortex, 
as  indicated  by  Krause,  are  not  increased. 

Lumbar  puncture  when  repeated  and  large  amounts  of  cerebro-spinal 
fluid  removed  may  sometimes  give  considerable  relief  in  epilepsy,  but 
this  procedure  is  inferior  to  the  operations  mentioned  above. 

EPILEPSIA  PARTIALIS  CONTINUA 

Under  this  name  Kojewnikoff  described  in  1894  a  special  form  of 
epilepsy  the  essential  feature  of  which  is  the  co-existence  of  typical  seizures 
and  persistent  partial  convulsions  between  the  seizures. 

Symptoms. — The  disease  may  begin  either  with  generalized  attacks 


JACKSONIAN    OR   FOCAL   EPILEPSY  1 29 

or  attacks  confined  to  one  limb,  to  the  face  or  to  one  half  of  the  body. 
These  attacks  are  typical  of  epilepsy.  In  the  intervals  between  the 
classical  seizures  frequent  involuntary  contractions  of  muscular  groups 
are  observed.  They  are  clonic  in  character.  Their  range  varies  from 
fibrillary  contractions  to  very  wide  movements.  They  always  occur  in 
the  same  part  of  the  body.  They  are  more  violent  immediately  before 
the  typical  epileptic  seizures,  but  after  the  latter  they  are  very  feeble. 
They  cannot  be  controlled  by  the  will.  They  increase  upon  emotion. 
During  sleep  they  decrease  and  may  disappear.  When  the  movements 
are  pronounced,  walking  is  difficult.  The  power  of  the  affected  side  is 
diminished;  atrophy  and  contracture  are  occasionally  observed.  The 
tendon  reflexes  are  exaggerated,  but  ankle-clonus  and  Babinski  sign  are 
absent.  Pain  is  sometimes  complained  of,  but  the  objective  sensibilities 
are  normal.  In  exceptional  cases  the  following  additional  phenomena 
have  been  observed:  aphasia  or  dysarthria,  facial  paresis,  acceleration  of 
pulse  and  respiration,  spasm  of  oesophagus,  involuntary  laughing  and 
crying. 

Course  and  Prognosis. — The  disease  runs  a  slow  course,  but  it  is  per- 
sistent and  interferes  with  the  patient's  occupation.  It  is  usually  aggra- 
vated after  an  attack  of  epilepsy.  The  outlook  is  serious.  Death  usually 
occurs  from  some  intercurrent  disease. 

Pathology  and  Pathogenesis. — Autopsies  of  cases  with  Kojewnikoff's 
syndrome  are  very  rare.  In  Osokine's  case  (Rev.  de.  med.  de  Moscou,  1908) 
a  portion  of  the  motor  cortex  on  the  side  opposite  to  the  seat  of  convulsions 
was  excised.  It  proved  to  be  a  meningo-encephalitis.  A  very  recent  case 
of  Kramholz  (/.  of  Nerv.  and  Ment.  Dis.,  1913)  with  autopsy  also  shows 
that  the  symptoms  were  due  to  encephalitis  with  a  sclerotic  process  in 
the  motor  cortical  region.  Choroschko  incriminates  also  the  optic 
thalamus  and  the  posterior  quadrigeminal  bodies.  The  pathogenesis  is 
still  debatable  in  view  of  the  paucity  of  anatomical  proofs. 

Etiology. — Infectious  diseases,  syphilis,  alcoholism,  trauma  have  been 
observed  as  immediately  preceding  the  onset  of  the  disease.  Men  are 
more  frequently  affected  than  women. 

Treatment.-^-Bromides,  nitroglycerine  are  the  only  drugs  which  may 
be  useful  in  decreasing  the  intensity  and  frequency  of  the  convulsions. 
Operative  procedures  give  better  results.  Horsley's  method  has  been 
used  with  great  satisfaction  (see  page  128). 


CHAPTER   VII 

•      APHASIA 

Under  the  old  conception  (Trousseau)  this  term  expressed  merely 
an  inability  to  express  ideas  in  words.  In  the  light  of  our  present  knowl- 
edge this  conception  became  naturally  broader  and  now  aphasia  means 
an  inability  to  communicate  ideas  not  only  in  words  but  with  any  other 
sign.  It  affects  therefore  two  faculties:  comprehension  and  expression. 
Consequently  there  are  two  kinds  of  aphasia:  (i)  aphasia  of  expression 
or  motor  aphasia  (Broca's  aphasia)  and  (2)  aphasia  of  comprehension  or 
sensory  aphasia  (Wernicke's  aphasia). 

I.  MOTOR  APHASIA 

Ideas  may  be  expressed  in  words  or  in  writing.  Loss  of  function  to 
articulate  words  is  called  aphemia,  to  write  agraphia. 

Aphemia. — Indivi duals  presenting  this  symptom  are  able  to  hear 
and  understand  when  spoken  to  (their  inner  or  intrinsic  language  is  intact) ; 
they  are  able  to  emit  sounds,  to  move  the  lips  and  tongue.  What  they 
have  lost  is  their  vocabulary;  otherwise  speaking,  the  faculty  to  articulate 
words. 

Aphemia  may  be  complete  and  incomplete.  In  the  first  case  the 
patient  cannot  articulate  even  one  syllable.  He  resembles  a  mute. 
More  frequently  his  vocabulary  consists  of  only  one  or  several  unin- 
telligible syllables  which  he  repeats  at  each  question.  In  the  second 
case  there  are  infinite  varieties:  here  the  vocabulary  may  be  reduced  to 
one  or  two  words,  which  the  patient  always  pronounces  and  repeats 
whenever  spoken  to;  or  to  a  whole  phrase.  In  mild  cases  only  certain 
words  are  wanting.  In  ordinary  cases  the  aphasic  gradually  regains 
his  lost  words  and  reeducation  may  help  considerably  in  this  respect. 
There  are  also  cases  which  are  absolutely  incurable.  In  individuals 
who  speak  several  languages,  aphasia  affects  first  the  acquired  languages 
and  when  improvement  takes  place,  the  patient  will  commence  to  speak 
his  native  tongue  long  before  the  foreign.  Incomplete  aphemia  may 
be  modified  under  certain  influences.  H.  Jackson  and  Trousseau  report 
cases  in  which  the  patients  during  an  emotion  (anger)  recovered  words 
which  they  otherwise  could  not  pronounce.     Some  aphasics  repeat  words 

130 


APHASIA  131 

spoken  close  to  their  ear.  Here  the  motor  speech  center  is  stimulated  by 
the  auditory  center.  Echolalia  is  thus  explained.  For  the  same  reason 
some  aphasics  while  singing  are  able  to  pronounce  words  which  otherwise 
they  cannot.  Visual  impressions  may  play  the  same  role  as  auditory. 
One  of  my  patients  could  pronounce  words  only  when  she  saw  them 
written.  As  to  the  anatomical  seat  of  aphemia,  according  to  the  classical 
view  it  is  the  posterior  portion  of  the  left  third  frontal  convolution 
(Broca's  region)  in  right-handed  and  the  same  portion  in  the  right 
hemisphere  in  left-handed  individuals.  (See  chapter  on  Localizations, 
and  Marie's  new  conception  of  Aphasia.) 

Agraphia. — The  patient  thus  affected  has  lost  the  faculty  of  expressing 
ideas  in  writing.  The  disturbance  of  this  function  may  present  various 
forms.  It  may  be  complete  when  the  patient  is  incapable  of  making  the 
slightest  sign  on  the  paper,  or  incomplete  when  only  one  word  or  certain 
words  can  be  written.  Sometimes  the  letters  are  irregularly  placed, 
sometimes  the  combination  of  letters  or  words  is  such  that  they  do  not 
correspond  to  the  idea  the  patient  wishes  to  express.  In  some  cases  the 
agraphia  affects  only  figures,  not  words;  in  others  only  copying  and  not 
spontaneous  writing;  in  still  others  the  patient  can  write  only  under 
dictation. 

In  paragraphia  the  patient  substitutes  to  the  right  words  words 
without  meaning.  The  agraphics  who  are  at  the  same  time  hemiplegics 
exercise  often  their  left  hand  for  writing,  but  instead  of  writing  normally 
from  left  to  right  they  write  from  right  to  left.  This  is  mirror 
writing.     It  resembles  a  writing  as  it  appears  through  a  mirror. 

Agraphia  without  motor  aphasia  (aphemia)  is  exceptionally  rare. 
It  is  sometimes  associated  with  other  forms  of  aphasia,  quite  frequently 
with  word-blindness.  The  function  of  word-seeing  (reading)  is  frequently 
necessary  for  writing  either  spontaneously  or  under  dictation;  if  this 
faculty  is  abolished  (alexia),  writing  is  impossible.  Copying,  however, 
is  possible  as  the  agraphic  is  able  to  imitate  letters  like  drawings 
even  without  knowing  their  meaning.  Indeed,  there  are  cases  on  record 
in  which  a  lesion  of  the  angular  gyrus  involved  also  the  faculty  of  writing. 
The  loss  of  function  of  word-hearing  may  also  cause  inability  to  write. 
The  very  frequent  association  of  agraphia  with  motor  aphasia  can  be 
easily  understood:  first  because  of  the  close  proximity  of  their  centers  (a 
lesion  of  one  can  hardly  escape  from  involving  the  other  center) ;  also,  be- 
cause of  the  well  known  fact  that  a  child  as  well  as  many  adults  pro- 
nounce words  or  articulate  them  while  they  write.  A  loss  or  impairment 
of  the  first  function  will  produce  a  disturbance  of  the  latter.  An  inde- 
pendent existence  of  agraphia  is  therefore  disputed.     However,   there 


132       '  APHASIA 

are  a  few  cases  on  record  in  which  there  was  no  other  disturbance  of 
speech  and  of  intelligence  and  still  the  faculty  of  writing  was  lost.  The 
anatomical  seat  of  the  function  of  writing  is  supposed  to  be  in  the  pos- 
terior portion  of  the  left  second  frontal  convolution.  This  question  is 
still  debatable. 

II.  SENSORY  APHASIA 

(a)  Word-deafness. — This  condition,  to  which  Kussmaul  in  1876  was 
first  to  call  attention,  consists  of  a  loss  of  the  faculty  of  understanding 
spoken  words.  The  patient  hears  the  voice,  as  his  general  hearing 
and  other  faculties  are  not  affected,  but  the  words  are  to  him  mere  sounds 
and  do  not  express  ideas.  Word-deafness  may  be  complete  (very  rarely) , 
when  the  patient  cannot  understand  one  word,  and  incomplete,  when 
only  some  words  are  properly  interpreted  by  him.  In  the  latter  case 
there  may  be  many  varieties  according  to  the  number  and  character  of  the 
words  understood.  Word-deafness,  when  it  is  pronounced,  is  associated 
with  other  speech  disorders.  In  ordinary  conditions  the  acts  of  speak- 
ing, reading  and  writing  are  all  dependent  on  inner  or  mental  hearing; 
it  is  the  inner  words  that  command  the  outer  words  (speaking)  and 
dictate  the  writing,  also  during  the  act  of  reading  the  mental  or  inner 
hearing  reproduces  the  words  read.  It  is  therefore  evident  that  visual 
images  are  intimately  associated  with  auditory  images  and  that  word- 
deafness  disturbs  the  faculties  of  speaking,  reading  and  writing. 

In  the  majority  of  cases  the  word-deaf  shows  some  disturbance  of 
motor  speech.  When,  for  example,  he  is  asked  a  question  in  writing 
his  verbal  answer  will  be  disturbed,  as  he  is  unable  to  hear  his  own  words. 
It  then  frequently  happens  that  the  patient's  entire  speech  becomes  in- 
comprehensible: either  he  uses  one  word  instead  of  another  (paraphasia) 
or  employs  unintelligible  words  (jargonaphasia).  Both  disorders  are 
then  observed  in  spontaneous  and  repeated  speaking  also  in  reading 
aloud,  because  the  patient  being  unable  to  hear  himself,  cannot  correct 
his  defective  speech.  Not  only  paraphasia  but  also  paragraphia  is  ob- 
served in  word-deafness.  The  patient  writes  like  he  speaks :  he  substitutes 
wrong  words  for  the  right  ones. 

There  is  a  variety  of  deafness  referable  to  music  (auditory  amusia),  in 
which  the  patient  is  unable  to  recognize  familiar  songs  or  unable  to 
distinguish  one  from  another.  There  is  also  a  variety  of  deafness  in 
which  not  words  but  figures  are  misunderstood  and  misinterpreted. 
Word-deafness  may  be  incurable,  but  in  the  majority  of  cases  with  the 
aid   of    reeducation    the   patient   regains   some   words.     Other   centers, 


APHASIA  133 

especially  the  visual,  render  great  assistance:  when  the  patient  is  often 
spoken  to,  he  learns  to  observe  the  movements  of  the  speaker's  lips 
and  thus  gradually  some  old  lost  verbal  images  return. 

The  anatomical  seat  for  word-deafness  is,  according  to  the  majority 
of  neurologists,  in  the  left  first  temporal  convolution  (see  "Localizations"). 

(b)  Word-blindness  or  Alexia.— It  was  first  described  by  Kussmaul 
in  1877.  It  is  characterized  by  an  inability  to  read  printed  or  written 
matter.  The  patient  lost  the  faculty  of  reading  even  his  own  writing, 
although  his  vision  and  intelligence  are  intact;  he  sees  the  form  of  the  letters 
but  not  the  idea  expressed  in  them.  Various  degrees  of  alexia  may  be 
present.  While  on  one  hand  it  may  be  complete,  so  that  not  one  letter 
is  discerned  by  the  patient  (letter-blindness  of  Kussmaul),  on  the  other 
one  or  several  words  or  only  syllables  may  be  seen.  There  may  be  blind- 
ness for  words,  but  not  for  letters;  the  patient  is  able  to  recognize  letters, 
but  not  able  to  understand  the  meaning  of  combinations  of  letters,  viz. 
syllables  or  words.  Similarly  to  word-deafness  we  find  here  also  varieties 
of  alexia  in  which  the  patient  is  blind  not  for  words  or  letters,  but  for 
figures  or  for  musical  notes. 

Patients  affected  with  alexia  present  usually  difficulty  in  spontaneous 
writing  as  the  writing  itself  consists  in  reality  of  copying  their  visual 
images,  but  the  agraphia  is  never  complete.  For  the  same  reason  writing 
under  dictation  is  impossible.  Copying  is  done  like  drawing.  When  the 
word-blindness  is  not  complete,  the  patient  may  be  able  to  recall,  visually, 
words  either  by  writing  or  by  hearing.  Thus  he  follows  sometimes  with  his 
finger  the  letters  which  he  cannot  see  with  his  eyes,  or  else  he  finds  the 
letters  or  words  when  he  hears  them  pronounced.  Graphic  or  auditory 
images  may  therefore  assist  in  recalling  visual  images. 

In  another  variety  of  word-blindness  the  patient  is  able  to  read  and 
copy  letters  and  words,  but  unable  to  comprehend  their  meaning.  It 
is  exactly  what  happens  when  thinking  of  another  subject  we  keep  on 
reading;  then  suddenly  we  notice  that  we  have  read  an  entire  page  or 
more  and  do  not  know  the  contents.  This  is  the  so-called  "psychic 
blindness." 

The  anatomical  seat  for  alexia  is  in  the-  left  angular  gyrus  (see 
"Localizations"). 

OTHER  FORMS  OF  APHASIA 

Aphasa  resulting  from  a  Lesion  beneath  the  Cortical  Centers  (Conduc- 
tion Aphasia  of  Lichtheim -Wernicke). — In  the  preceding  chapter  I  dis- 
cussed the  varieties  of  motor  and  sensory  aphasia  in  cases  when  the  lesion 


134  APHASIA 

lies  in  the  cerebral  centers,  viz.  in  the  cortex.  Anatomy  teaches  that 
that  various  portions  of  the  cortex  are  connected  with  each  other  by 
association  fibers  and  that  through  the  internal  capsule  (knee)  pass  motor 
fibers  whose  destination  is  to  supply  the  organs  of  phonation. 

A  lesion  situated  in  those  fibers,  viz.  beneath  the  cortex,  will  consti- 
tute the  so-called  (i)  "Transcortical  aphasia,"  or  pure  aphasias.  Similarly 
to  the  varieties  of  the  cortical,  the  transcortical  aphasia  also  presents  to 
the  four  main  types:  (i)  aphemia,  (2)  agraphia,  (3)  word-blindness  and 
(4)  word-deafness.  In  order  to  understand  the  mechanism  of  these  subcor- 
tical aphasias  it  is  necessary  to  bear  in  mind  the  anatomical  arrange- 
ment of  the  association  paths.  Supposing,  for  example,  that  a  lesion  has  in- 
terrupted the  connection  between  the  angular  gyrus  and  the  foot  of  the 
second  frontal  convolution,  viz.  between  the  center  for  word-reading  and 
the  writing  center.  It  is  easy  to  conceive  that  the  patient  will  be  able  to 
see  and  understand  (as  the  visual  centers  are  preserved),  to  read  aloud 
(as  the  fibers  connecting  the  angular  gyrus  with  Broca's  region  are  intact). 
The  patient  will  not  be  able  to  copy  what  he  sees  because  of  interrup- 
tion of  the  above  fibers,  but  he  will  be  able  to  copy  on]y  when  he  reads 
aloud,  because  of  the  integrity  of  the  fibers  connecting  the  auditory  verbal 
center  (first  temporal  convolution)  with  the  graphic  center. 

Another  example.  A  lesion  interrupts  the  association  fibers  between 
the  common  visual  center  and  the  center  for  word-reading  The  patient 
will  be  able  to  see  (as  the  common  visual  center  is  preserved),  to  copy 
(as  the  connection  between  the  common  visual  and  graphic  centers  is 
intact),  but  he  will  not  be  able  to  read;  the  letters  and  words  are 
incomprehensible.  These  are  the  so-called  pure  aphasias;  they  are  caused 
by  isolation  of  the  respective  speech-centers.  To  the  pure  motor  aphasia 
variety  Marie  gave  the  name  of  Anarthria  (see  below). 

The  two  examples  are  sufficient  to  understand  all  the  varieties  of 
aphasia  caused  by  a  lesion  of  the  sub-cortical  fibers.  It  is  necessary  only 
to  bear  in  mind  the  scheme  of  the  association  fibers. 

It  was  mentioned  above  that  the  internal  capsule  contains  motor 
fibers  for  speech.  A  lesion  of  these  fibers  will  produce  a  transcortical 
motor  aphasia.  In  this  case  the  disturbance  of  speech  will  not  be  similar 
to  that  of  a  lesion  of  Broca's  region.  It  will  consist  only  of  a  dysarthria 
or  anarthria  similar  to  that  found  in  lesions  of  peripheral  organs  of  the 
speech  or  in  bulbar  affections.  Here  the  cerebral  speech-function  is 
not  defective,  as  the  patient  has  the  power  of  communicating  his  thoughts 
in  every  way  but  the  words  are  unintelligible  (articulatory  defect). 

The  anatomical  distinction  between  the  cortical  and  sub-cortical  or 
transcortical  forms  of  aphasia  cannot  be  applied  with  such  a  degree  of 


APHASIA  135 

precision  to  the  clinical  picture;  otherwise  speaking,  is  there  any  clinical 
difference  between  the  two  groups  of  aphasia?  While  the  existence  of 
sub-cortical  aphasia  is  proven  beyond  doubt,  yet  clinically  it  is  impos- 
sible in  the  majority  of  cases  to  say  that  such  and  such  disturbance  of 
speech  is  due  to  a  lesion  of  the  cortical  centers  or  of  the  subjacent  fibers. 
Dejerine  and  Lichtheim,  however,  called  attention  to  a  few  differential 
points,  but  as  the  latter  present  some  objectionable  features  and  are  not 
accepted  by  the  majority,  it  is  unnecessary  to  go  into  details. 

(2)  Verbal  Amnesia. — Under  this  term  is  understood  an  inability 
to  recall  names.  When  the  disorder  is  only  slight,  the  individual  may 
after  a  certain  effort  recall  the  name  of  the  object;  he  will  do  it  either 
by  seeing  it  written  or  by  recalling  again  and  again  its  mental  image. 
In  complete  verbal  amnesia  all  the  efforts  are  in  vain;  he  will  not  be  able 
to  find  the  name  of  the  object.  According  to  Pitres,  this  form  of  aphasia 
is  caused  by  a  rupture  of  the  commissural  paths  which  connect  the  various 
centers  of  verbal  image  with  those  portions  of  the  cortex  on  which  the 
higher  mental  acts  depend.  It  is  therefore  also  a  transcortical  aphasia. 
On  the  other  hand,  cases  have  been  reported  by  Bianchi,  Mingazzini, 
Mills,  Pick  and  others  which  tend  to  show  that  the  inferior  parietal 
lobule  could  be  considered  as  the  center  of  verbal  amnesia. 

(3)  Optic  Aphasia  (Freund). — An  individual  thus  affected  recognizes, 
objects  placed  before  him  but  is  unable  to  name  them.  When,  however, 
he  palpates,  smells  or  tastes  them,  he  immediately  recalls  the  name. 
Physiologically  this  disorder  can  be  explained  either  by  interruption  of 
connection  between  the  visual  and  speech  center  or  by  an  insufficient 
excitation  of  the  cortex  by  the  optic  impression  of  the  unnamed  objects. 

(4)  Congenital  Aphasia  (Kussmaul). — It  is  not  the  result  of  a  local- 
ized central  lesion.  The  child  hears  and  understands  but  cannot  speak 
or  at  least  cannot  articulate  as  well  as  other  children  of  his  age.  The 
pathogenesis  is  obscure. 

General  Remarks  on  Aphasia. — The  preceding  study  of  motor  and 
sensory  speech  disturbances  may  lead  to  the  idea  that  each  form  of 
aphasia  is  independent  of  others.  In  reality  such  an  occurrence  is  an 
exception.  In  the  majority  of  cases  the  involvement  of  one  center 
affects  other  centers.  Aphemia,  for  example,  is  frequently  associated 
with  agraphia,  word-blindness  also  affects  the  faculty  of  writing, 
word-deafness  the  faculty  of  articulating  speech.  There  are  also  cases 
in  which  motor  aphasia  is  associated  with  sensory  aphasia;  all  forms  of 
speech  are  abolished.  The  aphasia  is  then  total.  The  latter  is  frequently 
coincident  with  right  hemiplegia.  Pathologically  however  (Gowers, 
Bastian,  Dejerine)  not  the  entire  speech  area  is  found  to  be  affected  in 


I36  APHASIA 

every  case.  Beduschi  (Encephale,  1910)  has  shown  that  total  aphasia 
occurs  almost  always  when  Wernicke's  zone  is  involved  together  with  the 
gyrus  supramarginalis.  In  the  white  substance  of  the  latter  lies  an  ar- 
cuate bundle  of  fibers,  through  which  pass  the  association  fibers  connect- 
ing the  visual  and  auditory  areas  with  the  motor  center  of  speech. 

The  mental  state  of  aphasic  individuals  deserves  mention.  It  is  usually 
affected.  The  enfeeblement  is  more  marked  in  the  sensory  than  in  the 
motor  form,  and  in  the  cortical  more  than  in  the  sub-cortical  type.  The 
degree  of  mental  impairment  depends  upon  the  extent  of  the  lesion  and 
varies  from  one  individual  to  another. 

Aphasia  in  left-handed  individuals  presents  the  same  characteristics 
as  in  right-handed  ones.  Pathologically  lesions  identical  with  those  de- 
scribed on  the  preceding  pages  have  been  found  in  the  right  hemisphere 
of  left-handed  individuals,  as  the  most  recent  studies  of  Mingazzini 
(Encephale,  1908),  and  of  Monakow  (Grosshirn  Pathologie,  1905)  proved 
it  conclusively. 

Latest  View  on  Aphasia. — It  was  just  mentioned  that  an  isolated 
involvement  of  any  one  of  the  speech-centers  is  a  great  rarity.  Indeed 
clinically  we  observe,  for  example,  in  the  motor  aphasia  of  Broca's  type 
not  only  loss  or  disturbance  of  spoken  language,  but  almost  always  some 
degree  of  alexia  and  agraphia;  also  some  disturbance  of  the  inner  (intrinsic) 
speech.  Pathologically  only  a  lesion  of  the  F3  is  held  accountable.  In 
the  sensory  type  of  aphasia  (Wernicke)  in  which  the  posterior  third  of  the 
T'  and  the  angular  gyrus  are  supposed  to  be  involved,  not  only  there  is 
a  defect  of  the  inner  speech  and  inability  to  understand  spoken  and 
written  words  but  there  is  also  a  marked  defect  of  motor  speech  (para- 
phasia and  jargon  aphasia).  There  have  been  cases  reported  in  which 
a  diagnosis  of  one  form  of  aphasia  was  made  during  life  and  at  autopsy 
lesions  corresponding  to  the  other  form  or  to  both  forms  simultaneously 
were  found.  In  view  of  the  complexity  of  the  subject  Marie's  claim  for 
a  reconsideration  of  the  problem  of  aphasia  becomes  a  matter  of  necessity 
and  the  following  is  the  new  view  held  by  Marie  and  his  followers. 

Marie's  View  on  Aphasia. — Basing  himself  on  clinicopathological 
records  of  a  large  number  of  cases  (Semaine  Medicale,  1906),  Marie  rejects 
the  following  features  of  the  old  classical  conception  of  aphasia,  viz.  the 
specificity  of  Broca's  center,  the  distinction  between  motor  and  sensory 
aphasias,  the  existence  of  pure  (sub-cortical)  forms  of  aphasia  and  finally 
the  existence  of  individual  speech  centers.  He  argues  that  there  is  only 
one  aphasia,  there  is  only  one  speech-center  localized  in  the  left  temporo- 
parietal lobe  which  is  at  the  same  time  the  specialized  intellectual  center 
but  not  the  center  for  sensory  images.     Word-deafness  and  word-blindness 


APHASIA 


137 


are  but  defects  of  the  special  intelligence  of  speech.  Intellectual  deficit 
is  the  main  characteristic  feature.  Broca's  aphasia  is  a  combination  of 
Wernicke's  aphasia  and  anarthria.  The  latter  is  caused  by  a  lesion  of 
a  quadrilateral  area  surrounding  the  lenticular  nucleus  (lenticular  zone)  ; 


Fig.   71. — Softening  in  Left  Hemisphere  Showing  Complete  Destruction  of  the 

Lenticular  Zone.     {Original.) 


its  limits  are:  anteriorly  a  vertical  plane  level  with  the  anterior  sulcus  of 
the  insula,  posteriorly  a  similar  plane  with  the  posterior  sulcus  of  the 
insula,  internally  the  lateral  ventricle,  externally  the  surface  of  the 
insula.  Posteriorly  the  lenticular  zone  is  in  connection  with  Wernicke's 
zone  (supra-marginal  gyrus;  angular  gyrus,  posterior  portions  of  the  T' 


138  APHASIA 

and  T")-  A  lesion  of  Wernicke's  zone  produces  aphasia,  a  lesion  of  the 
quadrilateral  area  produces  anarthria.  The  F3  which  lies  in  front  of  the 
quadrilateral  area  has  nothing  to  do  with  aphasia  (Fig.  71). 

Since  Marie's  original  contribution  various  observers  reported  ana- 
tomical cases  corroborating  his  views.  On  the  other  hand,  Dejerine  and 
others  brought  forward  facts  which  are  at  variance  with  the  new  con- 
ception of  aphasia.     The  subject  is  still  debatable. 

APRAXIA 

Under  this  name  is  understood  an  inability  to  use  the  limbs  for 
purposive  movements  in  spite  of  absence  of  paralysis  or  of  ataxia.  The 
apraxic  individual  understands  the  command  and  possesses  the  knowledge 
of  objects.  Nevertheless  he  uses  improperly  objects  presented  to  him 
and  executes  improperly  orders  given  him. 

Normally  to  fulfill  an  order  it  is  necessary  to  understand  it.  The 
execution  of  the  act  necessitates  the  following  conditions:  (1)  mental 
representation  of  the  act.  To  accomplish  the  act  it  is  necessary  to 
execute  a  series  of  movements.  Therefore  the  realization  of  the  princi- 
pal or  final  idea  is  associated  with  or  dependent  on  secondary  or  inter- 
mediary ideas.  The  latter  constitutes  the  kinetic  formula.  For  example, 
to  light  a  cigarette  necessitates  taking  out  a  match,  lighting  the  latter, 
put  the  cigarette  in  the  mouth,  etc.  To  accomplish  the  act  consequently 
one  must  have  a  mental  representation  not  only  of  the  final  act,  but  also 
of  secondary  acts.  (2)  The  psychic  conception  of  the  act  and  the 
kinetic  formula  must  be  followed  by  kinetic  images  which  means  beginning 
of  realization.  (3)  Finally  the  kinetic  images  are  followed  by  motor 
phenomena  which  finally  ends  in  execution  of  the  act.  Otherwise  speaking, 
there  are  three  elements  in  execution  of  an  act:  ideational  element,  trans- 
formation of  secondary  ideas  into  kinetic  images  and  a  motor  element. 
When  the  first  element  is  disturbed,  there  is  some  defect  in  the  superior 
mental  functions,  viz.  attention,  memory  and  association  of  ideas.  The 
patient  is  incapable  to  understand  the  details  of  the  act.  He  has,  therefore, 
ideational  apraxia.  When  the  second  element  suffers,  the  limbs  cannot 
obey  the  will.  The  patient  is  unable  to  transform  the  plan  of  the  act  into 
motor  images.  This  is  ideational-motor  apraxia  or  kinetic  apraxia  of 
Liepmann.  When  the  third  element  is  involved,  the  patient  is  incapable  to 
execute  correctly  the  movements.  Evidently  the  process  of  innervation 
is  inaccurate,  the  projection  centers  suffer.  This  is  motor  apraxia  or 
cortical  apraxia. 

Clinically  the  following  mainfestations  are  observed  (taking  for  a  type 


APHASIA  139 

the  ideational-motor  apraxia:  (1)  Substituted  movements.  Example: 
patient  opens  the  mouth  when  told  to  close  the  eyes.  (2)  Purposeless 
movements.  Example:  execution  of  an  act  having  no  relation  to  the  com- 
mand.    (3)  Absence  of  movements  without  regard  to  the  order. 

In  examining  for  apraxia  the  following  movements  are  to  be  investi- 
gated: (1)  Walking,  sitting  down  or  getting  up  (autokinetic  movements). 
(2)  Simple  movements:  close  the  eyes,  open  the  mouth,  put  out  the 
tongue,  raise  an  arm.  (3)  Show  the  right  or  left  eye  or  ear,  comb  the 
hair.  (4)  Salute  in  a  military  fashion,  make  the  movement  of  a  kiss.  (5) 
Make  the  movement  of  catching  a  fly.  (6)  Fill  a  glass  with  water,  seal  an 
envelope.  This  procedure  devised  by  Laignel-Lavastine  and  Boudon 
(Revue  Neurol.,  1909)  is  systematic  and  therefore  very  useful. 

The  study  of  apraxia  has  been  put  on  a  solid  scientific  basis  by 
Liepman  in  1900.  He  has  brought  forward  the  following  important 
anatomo-clinical  facts. 

Ideational-motor  apraxia  is  on  the  side  opposite  the  lesion,  but  it  may 
also  be  observed  on  the  same  side  when  the  lesion  is  in  the  left  hemisphere. 
The  preponderant  influence  of  the  left  hemisphere  on  the  functions  of 
speech  and  of  all  voluntary  movements  is  carried  through  the  corpus 
callosum.  By  means  of  the  latter  the  left  sensori-motorium  transmits 
its  influence  on  the  right  sensori-motorium.  To  obtain  apraxia  of  the  left 
hand  in  a  right  hemiplegic  it  is  necessary  to  have  a  sub-cortical  lesion 
affecting  simultaneously  the  projection  fibers  for  the  right  limbs  also  the 
fibers  of  the  corpus  callosum  destined  to  be  distributed  in  the  right 
senso-motorium  which  controls  the  left  limbs.  A  lesion  of  the  left  internal 
capsule  will  produce  a  right  hemiplegia  without  left  apraxia,  while  a 
lesion  only  of  the  corpus  callosum  will  result  in  left  apraxia  without  a 
right  hemiplegia  or  apraxia. 

Motor  apraxia  is  always  on  the  side  opposite  the  lesion.  Some 
authors  believe  that  it  is  due  to  superficial  changes  of  the  cortex. 

Ideational  apraxia  is  associated  with  a  suspension  or  a  real  dificit  of 
mental  faculties.  An  ideational  apraxia  occurring  in  a  hemiplegic  is  an 
important  indication  of  eventual  dementia. 


CHAPTER  VIII 

HEMIANOPSIA 

In  a  separate  chapter  will  be  described  ocular  disturbances  occurring 
in  diseases  of  the  entire  nervous  system.  Special  mention,  however, 
deserves  an  ocular  phenomenon  which  occurs  in  cerebral  affections  and 
which  it  is  important  to  recognize.     This  is  hemianopsia. 

Under  this  term  is  understood  a  complete  or  incomplete  blindness 
of  one  half  of  the  visual  fields.  When  the  corresponding  halves  of  both 
eyes  are  affected,  the  hemianopsia  is  homonymous  and  may  be  right 
or  left,  superior  or  inferior.  As  the  latter  two  are  comparatively  little 
known,  they  will  be  omitted  in  our  study.  In  the  majority  of  cases  the 
hemianopsia  is  lateral  homonymous.  When  the  blindness  of  the  visual 
fields  occurs  in  one  half  of  one  eye  and  in  the  opposite  half  of  the  other 
eye,  the  hemianopsia  is  then  heteronymous.  It  is  nasal,  when .  the 
right  half  of  the  left  and  the  left  half  of  the  right  visual  fields  are  blind. 
It  is  temporal,  when  the  right  half  of  the  right  and  left  half  of  the  left 
visual  fields  are  blind.  Anatomically  the  occipital  lobe  and  particularly 
the  calcarine  area  are  concerned  in  half  vision.  Embryological  investi- 
gation of  Flechsig  show  that  the  optic  radiations  which  end  in  the  calca- 
rine area  become  medullated  long  before  other  fibers  which  are  distributed 
in  the  occipital  lobe. 

In  order  to  understand  the  mechanism  of  hemianopsia  it  is  necessary 
to  bear  in  mind  the  entire  visual  pathway.  The  latter  consists  of:  (i) 
optic  nerves,  (2)  chiasma,^)  optic  tracts,  pulvinar,  external  geniculate 
bodies  and  anterior  quadrigeminal  bodies,  (4)  optic  radiations  and  cortex 
of  the  occipital  lobes.     (See  Anatomy  and  Localizations). 

The  decussation  of  the  optic  nerves  in  the  chiasma  is  not  complete, 
so  that  each  optic  tract  posteriorly  to  the  chiasma  contains  direct  fibers, 
which  pass  to  the  optic  nerve  of  the  same  side  and  become  distributed 
in  the  temporal  side  of  the  retina,  also  crossed  fibers,  which  are  distributed 
in  the  nasal  side  of  the  retina  of  the  opposite  side. 

With  these  anatomical  facts  in  view,  it  is  easy  to  conceive  the  mechan- 
ism of  hemianopsia.  A  lesion  situated  in  the  anterior  or  posterior 
angle  of  the  chiasma  will  interrupt  fibers  distributed  to  the  nasal  halves 
of  both  retinae  and  consequently  will  cause  blindness  of  both  temporal 
halves  of  the  visual  fields,  viz.  bitemporal  hemianopsia.     A  lesion  affecting 

14c 


HEMIANOPSIA  141 

the  right  optic  tract,  for  example,  will  involve  the  direct  fibers  going  to 
the  temporal  side  of  the  right  retina  and  the  crossed  optic  fibers  going  to 
the  nasal  side  of  the  left  retina;  the  symptom  will  be  a  homonymous 
left  hemianopsia,  otherwise  speaking  a  blindness  of  the  left  visual  field 
of  both  eyes.  The  same  form  of  hemianopsia  will  be  observed  in  lesions 
of  the  optic  radiations  and  of  the  occipital  lobe,  especially  in  the  calcarine 
fissure,  cuneus  and  lingual  lobule.  Although  the  anterior  quandri- 
geminal  body  and  external  geniculate  body  belong  to  the  optic  centers, 
there  are,  however,  no  exact  data  showing  that  a  lesion  of  these  bodies 
produces  a  homonymous  hemianopsia.  In  order  to  emphasize  the  rela- 
tion of  the  side  of  the  lesion  to  that  of  the  hemianopsia,  I  will  call  attention 
to  the  analogy  which  exists  between  this  phenomenon  and  the  motor 
and  sensory  disturbances  in  affections  of  one  cerebral  hemisphere.  Each 
hemisphere  is  in  control  of  the  opposite  side  of  the  body.  In  hemianopsia 
therefore  the  seat  of  the  lesion  is  on  the  side  opposite  to  the  blind  visual 
field. 

From  a  clinical  standpoint  two  problems  must  be  investigated.  First 
is  to  determine  the  hemianopsia.  Second  is  to  localize  it.  In  the  first 
case  the  patient  will  frequently  call  attention  to  the  lateral  loss  of  vision. 
Each  eye  must  be  examined  separately  and  in  the  majority  of  cases  the 
hemianopsia  is  easily  revealed.  The  next  question  is  to  determine  in 
what  portion  of  the  long  visual  pathway  lies  the  lesion.  This  can  be  as- 
certained from  the  reaction  of  the  pupil. 

As  is  well  known,  the  pupillary  fibers  of  the  optic  nerve  go  through 
the  anterior  quadrigeminal  body.  From  the  latter  a  system  of  fibers 
connect  the  former  with  the  nucleus  of  the  third  nerve  which  controls 
the  pupillary  sphincter,  which  is  located  in  the  gray  matter  of  the  aqueduct 
of  Sylvius.  A  lesion  therefore  in  front  of  the  quadrigeminal  bodies  will 
interrupt  the  connection  between  the  pupillary  fibers  of  the  optic  nerve 
with  the  nucleus  of  the  sphincter  of  the  pupil.  In  this  case  there  will 
be  a  homonymous  hemianopsia  with  no  response  of  the  pupil  when  a 
light  is  thrown  into  the  latter  on  the  blind  side  of  the  visual  field.  A 
lesion  placed  posteriorly  to  the  quadrigeminal  bodies  will  consequently 
not  interfere  with  the  pupillary  reaction.  The  inaction  of  the  pupil 
under  the  circumstances  just  described  is  known  as  "hemianopsic  pupil- 
lary reaction  "or  "Wernicke's  pupil." 

Homonymous  hemianopsia  has  this  characteristic  feature  that  the 
vision  central  is  always  preserved.  The  reason  of  it  lies  in  the  fact  that  the 
macula  of  each  eye  is  in  anatomical  relation  with  both  optic  tracts  and 
both  cortical  visual  centers  by  means  of  a  direct  and  crossed  bundle  of 
fibers.     Even  in  some  cases  of  double  hemianopsia  caused  by  bilateral 


142  HEMIANOPSIA 

lesions  in  the  occipital  lobes  central  vision  is  reported  to  be  normal. 
Such  were  the  cases  of  Forster  (Arch.  f.  Ophthalm.,  1890)  and  of  Beevor 
and  Collier  (Brain,  1904). 

Cortical  homonymous  hemianopsia  presents  this  peculiarity  that  it 
is  very  frequently  associated  with  hemiplegia  and  hemianesthesia,  be- 
cause the  destructive  lesion  in  the  occipital  lobe  and  optic  radiations  which 
causes  the  hemianopsia  frequently  extends  into  and  implicates  the  internal 
capsule.  When  the  lesion  affects  also  the  cortex  of  the  angular  gyrus, 
in  addition  to  hemianopsia  word-blindness  will  be  present. 

To  sum  up  the  study  of  homonymous  hemianopsia,  one  can  say  that 
it  is  caused  by  lesions  of  the  visual  pathway  between  the  chiasma  and 
the  occipital  cortex.  As  to  the  optic  nerves  in  front  of  the  chiasma,  in 
view  of  their  anatomical  composition  (see  above)  the  hemianopsia  may 
be  monocular,  when  only  a  half  of  one  optic  nerve  is  involved. 

Up  to  now  only  unilateral  hemianopsia  was  considered.  Hemi- 
anopsia may  be  double.  The  patient  is  then  totally  blind.  Such  cases 
are  usually  the  result  of  bilateral  cortical  lesions.  It  is  characterized 
by  the  integrity  of  the  fundi  and  absence  of  Wernicke's  pupillary  reaction. 
Curiously  enough  the  central  vision  is  preserved  or  rapidly  reestablished, 
probably  because  of  the  relation  of  the  macula  lutea  to  both  cortical 
visual  zones  (see  above). 


CHAPTER  IX 

TUMORS  OF  THE  BRAIN 

The  brain  is  a  frequent  seat  of  growths  in  children  and  adults.  They 
may  originate  in  the  cerebral  substance,  in  the  meninges  or  in  the  cranium 
itself.  In  view  of  the  great  resistance  of  the  skull  to  expansion  of  intra- 
cranial neoplasms,  the  brain  is  always  under  pressure  irrespective  of  the 
point  of  origin  of  the  tumor.  Disturbances  of  mechanical  nature  are 
therefore  most  prominent  in  tumors  of  the  brain. 

Pathology. — In  studying  the  pathology  two  elements  are  to  be  con- 
sidered: (i)  the  tumor  itself  and  (2)  the  condition  of  the  cerebral  tissue. 

(a)  Varieties  of  Tumors. — In  order  of  their  frequency  they  are:  glioma- 
tous,  sarcomatous,  tubercular,  syphilitic  (gumma),  endotheliomatous, 
vascular,  cystic  and  parasitic  (echinococcus  and  cysticercus) ,  carcinom- 
atous, cholesteatomatous,  osteomatous. 

Glioma. — It  is  a  soft  and  very  vascular  tumor.  It  is  never  encapsu- 
lated, but  continuous  with  the  neighboring  cerebral  tissue:  the  brain  sub- 
stance is,  so  to  speak,  infiltrated  with  the  soft  gliomatous  tissue.  For 
this  reason  the  exterior  aspect  of  the  cortex  undergoes  very  little  change; 
there  is  only  a  distinct  softness  of  the  brain.  The  most  frequent  seat  of 
glioma  is  the  white  substance  beneath  the  cortex. 

Histologically  it  is  essentially  composed  of  neuroglia  tissue.  The 
latter  presents  its  characteristic  appearance,  viz.  cells  with  their  large 
nuclei,  isolated  or  in  masses.  When  in  addition  to  them  there  are  also 
connective  tissue  elements,  the  tumor  is  called  gliosarcoma.  In  rare 
cases  (probably  congenital)  a  hyperplasia  of  the  cortical  elements  is 
seen  besides  abundant  neuroglia  and  vascular  tissue.  It  is  called  the 
neuro-glioma.  Glioma  is  seen  more  frequently  in  middle  life  than  in 
old  age. 

Sarcoma. — It  is  a  soft  tumor,  but  distinctly  harder  than  glioma.  It 
presents  this  characteristic  feature,  that  there  is  a  sharply  defined  line  of 
demarcation  between  it  and  the  brain  tissue  and  it  is  therefore  easily 
separable  from  the  latter.  It  is  frequently  encapsulated.  It  is  reddish 
and  of  spheric  form.  Histologically  it  consists  •  essentially  of  round  or 
spindle  cells;  it  is  not  vascular. 

In  the  majority  of  cases  sarcomata  originate  in  the  dura-mater,  peri- 
osteum or  skull.     They  develop  frequently  at  the  base  of  the  brain. 

i43 


144  TUMORS    OF    THE   BRAIN 

There  is  a  variety  of  sarcomata  characterized  by  a  proliferation  of  con- 
nective tissue.  They  are  called  fibro-sarcomata.  Their  multiplicity  and 
localization  all  over  the  central  and  peripheral  nervous  system,  particularly 
in  the  cerebello-pontine  region,  also  in  the  roots  of  the  spinal  cord  and 
cauda  equina,  are  the  characteristic  features  of  these  tumors.  Sarcoma 
occurs  particularly  in  middle  life.  A  very  malignant  form  is  melanotic 
sarcoma  which  is  a  secondary  tumor. 

Tubercular  Tumor. — Solitary  tubercles  belong  to  the  most  frequent 
growths  of  the  brain.  Unlike  glioma  they  have  a  remarkable  tendency 
to  become  encapsulated.  They  are  generally  of  the  size  of  a  cherry  and 
of  a  spheric  form.  Their  usual  seat  is  in  the  most  vascular  areas,  viz. 
in  the  fissure  of  Sylvius,  at  the  base  of  the  brain,  in  the  vicinity  of  the 
pons  and  in  the  interpeduncular  space.  The  most  common  seat  is  around 
the  Pacchionian  bodies  (see  Anatomy).  They  are  found  more  frequently 
near  the  meninges  than  within  the  cerebral  tissue.  The  cerebellum  is  a 
favorite  seat  of  tubercles  in  children.  There  is  frequently  more  than 
one  tumor.  On  section  no  vessels  will  be  seen  in  the  center,  which  is 
in  a  caseous  state.  The  granular  tissue  of  which  the  tubercule  is  com- 
posed is  formed  at  the  expense  of  the  perivascular  sheaths  and  of  the 
neuroglia  tissue.  The  tubercle  bacillus  is  very  frequently  found.  Tuber- 
cular tumors  may  produce  tuberculous  meningitis. 

Syphiloma  (Gumma). — These  tumors  are  frequently  found  in  adults. 
Small  in  size  (chestnut),  irregular  and  nodular  in  shape,  firm  in  some  parts 
and  soft  in  others,  they  are  most  frequently  located  at  the  base  of  the 
brain  and  quite  often  also  in  the  cortex,  especially  in  the  anterior  portions 
of  the  hemispheres.  At  the  base  they  inevitably  involve  the  cranial 
nerves  or  the  large  blood  vessels,  the  obliteration  of  which  naturally  leads 
to  a  softening  of  cerebral  tissue.  The  meninges  are  usually  involved 
("inflammation,  adhesion)  so  that  it  is  difficult  to  tell  where  the  growth  origi- 
nated: in  the  brain  tissue  or  membranes.  There  are  often  several  gum- 
mata.  Very  small  gummata  are  sometimes  found  around  the  blood 
vessels;  they  resemble  then  miliary  tubercles,  but  the  latter  are  generally 
extremely  small.  On  section  they  present  irregular  cheesy  spots,  between 
which  is  seen  fibrous  tissue.     Like  the  tubercle,  syphiloma  is  not  vascular. 

Endothelioma  usually  originates  in  the  dura  and  is  single.  It  occurs 
at  any  age. 

Vascular  Tumors. — Angiomata  are  rare,  but  aneurisms  are  not  very 
rare.  They  are  usually  found  on  the  bents  or  curves  which  the  arteries 
form  in  their  course.  Syphilis  is  probably  the  cause  of  cerebral  aneurisms. 
Statistics  show  that  the  basilar  artery  is  the  most  frequently  affected  and 
the  next  in  frequency  is  the  middle  cerebral. 


TUMORS    OF    THE   BRAIN  145 

Cystic. — Besides  cystic  formations  developed  in  place  of  old  hemor- 
rhagic foci  or  areas  of  softening  (see  Apoplexy) ,  there  are  also  some  which 
develop  in  connection  with  sarcomata  or  gliomata.  When  any  of  these 
tumors  breaks  down  and  the  debris  are  carried  off,  a  cavity  surrounded  by 
walls  composed  of  the  elements  of  the  growth  takes  its  place  and  thus  a 
cyst  is  formed.  These  formations  usually  occupy  the  ventricular  cavities 
and  are  also  found  in  the  cerebellum. 

Occasionally  the  cerebral  substance  or  the  meninges  are  the  seat  of 
cysts  containing  parasites,  viz.  cysticercus  and  echinococcus.  The 
cysticercus  is  found  in  the  meninges  and  particularly  in  the  area  of  the 
perforated  spaces.  The  latter  fact  is  the  reason  of  the  ocular  phe- 
nomena observed  during  life.  The  echinococcus  is  observed  especially 
in  certain  countries,  Australia  for  example.  It  usually  forms  adhesions 
with  the  meninges  and  it  may  be  eliminated  through  the  natural  openings 
of  the  cranium. 

Carcinoma.— In  the  majority  of  cases  it  is  secondary  to  carcinoma  of 
other  organs  of  the  body.  It  is  comparatively  frequent  in  the  ventricular 
cavities  or  in  the  walls  of  the  ventricles.  It  is  vascular  and  rarely  encap- 
sulated. It  is  usually  multiple.  It  occurs  mostly  in  aged  people.  Cho- 
leastoma  and  Osteoma  are  very  rare.  The  former  is  easily  recognizable 
by  its  brilliant  appearance. 

(b)  Effect  of  Tumors  on  Brain  Tissue. — Cerebral  tumors  have  a 
direct  effect  upon  the  neighboring  nervous  tissue  and  a  distant  effect 
upon  all  parts  of  the  brain.  Direct  pressure  produces  displacement  of 
the  brain  toward  the  point  of  least  resistance,  which  is  usually  the  ven- 
tricles, flattening  of  the  convolutions  and  destruction  of  nerve  elements. 
The  meninges  are  tense.  The  area  of  softening  which  is  usually  seen 
around  the  neoplasm  is  due  to  the  destruction  of  the  nerve  tissue.  The 
latter  process  is  an  irritative  process,  which  eventually  leads  to  an  in- 
flammation. Distant  pressure  is  sometimes  the  cause  of  meningitis, 
which  is  observed  in  the  course  of  brain  tumors.  The  cerebro-spinal 
fluid  is  habitually  increased,  so  that  the  entire  brain  is  wet  (oedema)  and 
internal  hydrocephalus  may  be  produced.  The  latter  condition  is 
particularly  marked  in  tumors  situated  in  the  vicinity  of  the  openings 
connecting  the  ventricles,  as,  for  example,  near  the  middle  lobe  of  the 
cerebellum  or  the  quadrigeminal  bodies.  When  pressure  from  the  tumor 
or  from  the  hydrocephalus  is  produced  toward  the  base  of  the  brain,  the 
cranial  nerves  are  involved. 

The  increased  intracranial  pressure  produces  an  obstacle  to  the  free 
cerebral  circulation  thus  causing  an  increase  of  the  intracranial  venous 
pressure.     The  latter  has  a  direct  effect  on  the  cerebro-spinal  fluid  thus 


146 


TUMORS    OF    THE  BRAIN 


rising  its  pressure.  As  the  increase  of  this  fluid  is  not  only  in  the  cranium 
but  also  in  the  spinal  canal,  the  arachnoid  of  the  latter  becomes  distended 
and  the  posterior  nerve-roots  are  thus  over-extended.  Degenerative 
changes  of  these  roots  and  of  the  posterior  columns  are  not  infrequently 
met  with  in  cerebral  tumors  (Fig.  72). 


Fig.  72. — Tremor  (Round-cell  sarcoma.)     Wedged  in  between  the  L.  Frontal  and 
Temporal  Lobes,  Penetrating  the  Lateral  Ventricle.     (Original.) 


Symptoms. — They  are  (A)  general  and  (B)  local. 

A.  The  general  symptoms  are  common  almost  to  all  forms  of  tumors 
irrespective  of  their  seat  and  nature.  They  are:  headache,  vomiting, 
vertigo,  optic  neuritis,  general  convulsions,  insomnia,  mental  failure  with 
depression. 

Headache. — This  is  one  of  the  earliest  symptoms.     It  has  no  definite 


TUMORS    OF   THE  BRAIN  147 

seat  and  it  rarely  corresponds  to  the  seat  of  the  tumor.  It  is  deep  and 
persistent;  it  is  usually  continuous  and  sometimes  excruciating.  It  may 
become  aggravated  upon  slight  pressure  with  the  hand  (which  suggests 
an  involvement  of  the  skull  or  of  membranes)  or  upon  a  slight  motion  of 
the  head,  upon  coughing  or  forced  respiration.  It  is  usually  progressive 
in  intensity  in  the  course  of  the  disease  and  not  relieved  by  ordinary 
drugs.  In  many  cases  it  starts  in  the  morning  and  then  it  is  accompanied 
by  vomiting.  Sometimes  it  is  worse  at  night  and  then  syphilis  should  be 
thought  of,  although  specific  infection  is  not  invariably  the  case.  The 
cause  of  the  headache  lies  probably  in  the  irritation  of  the  meninges 
due  to  increased  intracranial  pressure.  Vomiting  is  characteristic  by 
the  facility  with  which  it  is  brought  on  and  by  its  occurrence  irrespective 
of  food.  This  is  the  so-called  "cerebral  vomiting."  They  usually 
coincide  with  periods  of  exacerbation  of  headache.  Vertigo  is  constant, 
but  particularly  marked  in  cerebellar  tumors. 

Optic  Neuritis. — Circulatory  disturbance  of  the  retina  is  a  very 
frequent  occurrence  in  cerebral  growths  (80  per  cent.).  It  may  result  in 
optic  neuritis  or  choked  disc.  The  papilla  is  in  a  stage  -of  cedematous 
infiltration  with  venous  stasis  very  probably  as  the  result  of  increased  intra- 
cranial tension.  Neuritis  is  rarely  unilateral.  One-sided  optic  neuritis 
is  more  frequently  met  with  in  cerebral  than  in  cerebellar  tumors  and 
when  it  occurs  it  is  frequently  on  the  side  of  the  tumor;  it  is  therefore  of 
localizing  importance.  A  certain  degree  of  optic  neuritis  may  exist  for 
weeks  and  months  without  appreciable  impairment  of  sight.  When  the 
neuritis  becomes  progressive  or  choked  disc  is  beginning  to  form,  limitation 
of  visual  field  will  be  noticed  and  gradual  blindness  will  develop.  The 
natural  and  ultimate  consequence  of  optic  neuritis  is  white  atrophy  and 
consequently  amaurosis.  In  a  small  percentage  of  cases  loss  of  vision 
occurs  before  atrophy  of  the  optic  nerve  develops.  Cushing,  Bordley 
and  Heuer  have  recently  shown  that  alterations  of  color  fields  (inversion) 
frequently  occur  in  intracranial  tumors  as  an  early  manifestation. 

The  following  peculiarities  were  noticed  by  them:  (1)  A  tumor  may 
advance  slowly  and  be  unaccompanied  by  cerebral  oedema  or  other 
pressure  symptoms;  distortion  of  the  color  fields  (dyschromatopsia)  may 
be  the  earliest  indication  of  the  latter  and  observed  on  the  side  opposite 
to  the  lesion.  (2)  Unilateral  dyschromatopsia  accompanied  by  an 
incipient  degree  of  choked  disc  may  be  on  the  side  of  the  lesion  and  is 
therefore  of  localizing  value;  if  dyschromatopsia  is  bilateral,  the  higher 
degree  of  it  is  on  the  side  of  the  lesion.  (3)  Dyschromatopsia  is  a  more 
delicate  manifestation  of  intracranial  pressure  than  papillcedema. 

Convulsions. — Epileptiform  generalized  convulsions  are  not  infrequent 


148  TUMORS    OF   THE  BRAIN 

in  brain  tumors.  They  are  due  to  an  irritation  of  the  cerebral  tissue. 
They  usually  appear  unexpectedly,  but  never  at  the  beginning  of  the  dis- 
ease. They  have  the  character  of  genuine  essential  epilepsy,  viz.  general- 
ized. According  to  Hirt's  statistics  this  form  of  epilepsy  occurs  in  50 
per  cent,  of  cases  of  brain  tumor.  There  are  cases  on  record  in  which 
epilepsy  was  for  a  long  time  the  only  symptom  of  brain  tumor.  Conse- 
quently in  a  given  case  of  epilepsy  occurring  late  in  life  without  an  apparent 
cause,  cerebral  neoplasm  should  be  thought  of.  When  the  convulsions 
are  focal  but  not  generalized,  they  have  a  localizing  value  (see  below). 

Insomnia.- — Sleeplessness  is  quite  frequent.  It  is  probably  due  to 
the  continuous  severe  headache.  It  can  also  be  explained  on  the  basis 
of  circulatory  disturbances  in  the  brain.  It  is  more  frequent  in  adults 
than  in  children. 

Mental  Disturbances. — Hebetude,  apathy,  indifference,  impairment 
of  memory,  depression  or  else  irritability  are  all  symptoms  which  may  be 
encountered.  Occasionally  symptoms  of  true  dementia  with  hallu- 
cinations or  delusions  may  develop.  The  latter  form  is  particularly 
present  in  tumors  of  the  frontal  lobe.  Stupor  is  usually  a  late  symptom. 
Somnolence  is  frequent.  Whenever  the  patient  is  free  from  suffering, 
he  goes  to  sleep  In  a  boy  of  twelve,  with  a  cerebellar  neoplasm,  I  ob- 
served an  unusual  tendency  to  sleep,  so  that  during  the  last  eight  days  he 
slept  continuously  except  for  a  few  minutes  during  the  day  to  take  nourish- 
ment. In  the  last  twenty-four  hours  he  could  not  be  aroused  and  gradu- 
ally expired.  Intense  somnolence  is  also  observed  in  tumors  of  pituitary 
body.  In  tumors  of  corpus  callosum  Raymond  observed  eccentricity  of 
actions,  impairment  of  memory  and  apparent  integrity  of  intelligence. 

B.  Local  Symptoms.  Tumors  of  the  Antero-superior  Portion  of  the 
Frontal  Lobe. — While  mental  disturbances  may  occur  in  tumors  of  any 
portion  of  the  brain  (see  preceding  chapter,  also  chapter  on  Localization 
of  Center  of  Intelligence),  it  is  nevertheless  averred  that  they  are  more 
frequent,  more  precocious,  more  pronounced  in  tumors  of  the  prefrontal 
area  than  in  those  of  any  other  portion  of  the  brain.  Consequently  when 
they  appear  early  they  have  a  localizing  value.  As  to  the  character  of 
the  psychic  disturbances  it  is  impossible  as  yet  to  draw  positive  conclu- 
sions. The  carefully  collected  records,  however,  show  that  the  main 
symptoms  are:  change  of  character,  inability  to  concentrate  attention, 
incoherence  in  speech,  deficiency  of  orientation  and  of  discrimination. 
Jastrowitz  (Deut.  med.  Wochenshr.,  1888)  described  a  special  mental 
manifestation  which  he  called  "moria."  It  consists  of  an  emotional 
gaiety,  of  an  unusually  lively  spirit.  The  author  claims  that  he  observed 
it  frequently  in  prefrontal  growths.     (See  also  my  article  on  the  Function 


TUMORS    OF    THE  BRAIN  149 

of  the  Prefrontal  Lobes  in  /.  Amer.  Med.  Ass.,  1907.)  In  tumors  of  this 
area  are  sometimes  also  observed  paralysis  or  localized  convulsions  on 
the  side  opposite  to  the  lesion.  These  symptoms  occur  when  the  tumor  is 
deep  seated,  so  that  it  involves  the  short  commissural  fibers  connecting 
the  prefrontal  lobe  with  the  motor  area  or  when  the  irritation  of  the  pre- 
frontal cortex  extends  backward  to  the  cortex  of  the  motor  area.  In  such 
cases  there  will  be  changes  in  the  deep  and  superficial  reflexes  (see  chapter 
on  Reflexes).  Usually  in  prefrontal  tumors  there  is  only  a  paresis  of 
the  opposite  side  of  the  body.  In  cases  of  localized  convulsions  a  paretic 
condition  will  follow  on  the  affected  side.  G.  Stewart  observed  in  a 
few  cases  a  fine  intention  tremor  of  the  head  on  the  side  of  frontal  tumor. 
The  same  author  calls  attention  to  a  loss  or  diminution  of  the  abdominal 
reflex  on  the  side  opposite  to  the  tumor. 

Tumors  of  the  Antero-inferior  Portion  of  the  Frontal  Lobe. — When 
the  second  frontal  convolution  is  involved  the  characteristic  focal  symp- 
tom is  agraphia.  When  the  third  frontal  convolution  is  affected,  the 
symptoms  will  be  motor  aphasia.  It  is  to  be  remembered  that  in  right- 
handed  people  aphasia  and  agraphia  will  be  caused  by  tumors  of  the 
above  area  in  the  left  hemisphere,  while  in  left-handed  people  the  lesion 
will  be  in  the  right  hemisphere  (see  chapter  on  Localizations).  Tumors 
of  the  frontal  lobe  compressing  the  olfactory  bulb  or  situated  on  the 
basal  surface  of  the  frontal  lobe  are  associated  with  loss  of  smell  in  the 
nostril  on  the  side  of  the  tumor. 

Tumors  of  the  Motor  Area.— In  view  of  the  motor  function  of  this 
area  (see  Localizations)  a  tumor  developing  gradually  and  irritating 
it  will  give  rise  to  motor  symptoms  in  the  form  of  tonic  or  clonic  convul- 
sions limited  to  one  side  of  the  body.  They  are  known  as  focal  or  Jack- 
sonian  epilepsy  (see  this  chapter)  In  this  form  of  epilepsy  consciousness 
is  usually  intact.  The  seat  of  the  spasm  (face,  arm  or  leg)  will  depend 
upon  the  seat  of  the  tumor  in  the  three  distinct  portions  of  the  motor  area 
(see  Localizations).  It  happens  not  infrequently  that  the  convulsions 
commencing  in  one  arm,  in  one  leg,  in  one-half  of  the  face  or  in  the  distal 
end  of  one  limb  (thumb,  fingers,  toes)  spread  rapidly  to  the  rest  of  the 
body  on  the  same  side  or  to  the  entire  body.  Sometimes  convulsions 
affecting  one-half  of  the  body  are  preceded  by  a  paresthesia  in  one  limb, 
as,  for  example,  a  tingling  sensation,  numbness  or  pins  and  needles. 
These  strictly  localized  phenomena  are  of  utmost  importance  for  diagnos- 
tic purposes :  the  order  of  extension  of  the  spasm  is  invaluable  for  localizing 
the  initial  irritation  and  consequently  the  seat  of  the  tumor.  The  pres- 
ence of  a  sensory  aura  indicates  the  seat  of  the  tumor  in  the  neighborhood, 
of  the  ascending  parietal  or  the  parietal  convolutions. 


150  TUMORS    OF    THE  BRAIN 

Tumors  of  the  motor  area  produce  also  paralytic  symptoms,  but 
they  usually  come  on  later  in  the  course  of  the  disease.  They  are  generally 
monoplegic,  but  may  become  hemiplegic.  Convulsions  are  usually 
associated  with  paralysis,  but  there  are  cases  of  brain  tumor  with  a 
gradually  developing  paralysis  without  convulsions  A  progressive 
paralysis,  viz.  monoplegia  slowly  developing  into  hemiplegia,  is  almost 
pathognomonic  of  cerebral  tumor.  In  such  cases  the  tumor  is  situated 
in  the  sub-cortical  white  matter,  viz.  in  the  motor  fibers  uniting  the 
cortical  motor  area  with  the  internal  capsule.  When  the  tumor  originat- 
ing in  the  sub-cortical  tissue  continues  to  grow  and  reaches  the  cortex, 
the  patient  will  begin  the  disease  with  paralytic  symptoms  and  later 
develop  convulsions.  Hemiplegia  may  set  in  suddenly.  It  is  then  an 
indication  of  a  hemorrhage  into  the  tumor  or  in  the  surrounding  tissue. 

In  tumors  of  the  motor  area  with  paralytic  symptoms  (hemiplegia 
or  monoplegia),  the  usual  signs  of  paralysis  of  cerebral  origin  are  ob- 
served (see  Hemiplegia).  In  cases  of  focal  epilepsy  without  paralysis 
the  reflexes  of  the  affected  side  are  usually  increased  and  especially 
immediately  after  each  attack.  The  paradoxical  reflex  (see  chapter  on 
Hemiplegia)  is  particularly  persistent  immediately  after  as  well  as  between 
the  individual  attacks.  This  reflex  therefore  is  valuable  as  it  is  indi- 
cative of  a  localized  cortical  irritation. 

Tumors  of  the  Ascending  Parietal  Convolution  and  of  the  Parietal 
Lobe. — The  most  conspicuous  symptoms  are  disturbances  of  general 
sensations  (hemianaesthesia)  and  particularly  of  muscular  sense  on  the 
opposite  side  of  the  body.  The  impairment  of  sensations  is  more  marked 
in  the  distal  than  in  the  proximal  ends  of  the  limbs.  For  details  and  for 
the  study  of  astereognosis  the  reader  is  referred  to  the  chapter  on 
Localizations.  A  tumor  seated  in  the  posterior  portion  of  the  inferior 
parietal  lobule  (angular  gyrus)  of  the  left  hemisphere  will  give  rise  to  word- 
blindness  (alexia).  In  case  a  parietal  tumor  will  encroach  upon  the 
motor  zone,  in  addition  to  the  above  symptoms,  there  will  be  also  paralysis. 
In  parietal  tumors  Jacksonian  fits  occur,  but  always  commencing  with  a 
sensory  aura.  If  the  parietal  tumor  extends  backward  so  that  the  occipital 
lobe  becomes  involved,  there  will  be  also  hemianopsia. 

Tumors  of  the  Occipital  Region. — The  chief  focal  symptom  of  tumors 
in  this  area  is  lateral  homonymous  hemianopsia.  For  details  see  chapter 
on  Localizations  and  Hemianopsia.  Frequently  there  are  also  subjective 
visual  disturbances,  such  as  flashes  of  light,  of  colors.  They  are  due  to 
an  irritative  state  of  the  cerebral  tissue.  When  the  tumor  is  on  the 
lower  surface  of  the  occipital  lobe  so  that  pressure  on  the  cerebellum 
will  be  present,  cerebellar  manifestations  will  follow.     When  a  tumor  of 


TUMORS    OF   THE   BRAIN  151 

the  occipital  lobe  extends  forward  so  that  it  encroaches  on  the  angular 
gyrus,  in  addition  to  the  hemianopsia  there  will  also  be  alexia.  Convul- 
sions, general  or  focal  (on  the  opposite  side)  occasionally  occur.  Paralysis 
on  the  opposite  side  also  occurs  when  the  tumor  extends  forward. 

Tumors  of  the  Temporal  Lobe. — The  function  of  hearing  is  associated 
with  the  temporal  lobe  (see  Localizations).  A  tumor  of  the  left  first  tem- 
poral lobe  in  right-handed  people  and  of  the  right  first  temporal  lobe  in 
left-handed  people  gives  rise  to  word -deafness  (see  chapter  on  Aphasia). 
In  a  specimen  exhibited  by  me  before  the  Philadelphia  Neurological  and 
Pathological  Societies  in  April  and  May,  1906,  the  left  first  temporal 
convolution  was  extremely  thin  and  narrow.  The  patient  presented 
during  life  word-deafness:  he  heard  sounds,  but  could  not  understand 
spoken  words. 

Tumors  of  the  apex  of  the  temporal  lobe  are  believed  to  cause  dis- 
turbances of  taste  and  smell. 

Hughling  Jackson  in  1876  described  attacks  consisting  of  a  "dreamy 
state"  preceded  by  peculiar  sensations  of  taste  or  smell.  During  the 
attack  the  patient  has  a  feeling  of  unreality  and  recalls  events  that  hap- 
pened long  ago.  At  the  same  time  he  keeps  on  moving  his  mouth  and 
jaws  as  if  he  chews.  These  seizures  are  called  "uncinate  fits,"  and  are 
observed  in  tumors  of  the  tip  of  the  temporo-sphenoidal  lobe. 

Tumors  at  the  Base  of  the  Brain. — In  view  of  the  presence  of  the 
cranial  nerves  at  the  base  of  the  brain  (see  Anatomy),  the  symptomatology 
of  basal  tumors  is  somewhat  complex.  The  characteristic  feature  of  such 
conditions  is  crossed  or  alternating  paralysis.  It  consists  of  hemiplegia 
affecting  the  side  of  the  body  opposite  to  the  side  of  the  lesion  and  of  a 
palsy  of  one  or  more  cranial  nerves  on  the  side  of  the  lesion.  It  finds  its 
explanation  in  the  fact  that  a  tumor  lying  on  one  side  involves  the  motor 
and  sensory  fibers  which  have  not  yet  decussated  (see  Anatomy),  also 
the  superficial  origin  of  the  cranial  nerves  which,  of  course,  do  not 
undergo  decussation  at  the  base.  Should  the  tumor  occupy  the  middle 
of  the  base,  bilateral  paralysis  will  necessarily  ensue.  It  is  superfluous 
to  describe  the  symptoms  of  involvement  of  each  cranial  nerve.  The 
deductions  present  no  difficulty,  if  their  anatomy  and  physiology  are 
taken  into  consideration.  For  example,  a  tumor  in  one  of  the  crura 
involves  the  third  nerve;  a  tumor  in  the  upper  half  of  the  pons  involves 
the  third  and  fifth  nerves.  In  the  first  case  there  will  be  a  crossed  hemi- 
plegia with  external  strabismus  or  ptosis  on  the  side  of  the  lesion;  in  the 
second  case,  also  crossed  hemiplegia  with  third  nerve  symptoms  and 
sensory  disturbances  of  the  face  on  the  side  of  the  lesion.  See  also  chapter 
on  Bulbar  Paralysis.     Tumors  of  the  very  anterior  portion  of  the  basal 


152 


TUMORS    OF    THE  BRAIN 


surface  of  the  brain,  viz.  of  the  inferior  surface  of  the  frontal  lobes,  will 
give  rise  to  early  visual  disturbances  and  to  olfactory  manifestations. 
They  find  their  explanation  in  compression  of  the  optic  and  olfactory 
nerves. 

Tumors  of  the  Quadrigeminal  Bodies. — In  view  of  the  anatomical 
relation  of  corpora  quadrigemina  to  the  cerebellum  and  to  the  motor 
nerves  of  the  eyes,  the  symptoms  will  be  those  observed  in  cerebellar 


Fig.  73' — Tumor  of  the  Basal  Ganglia  (Mixed-cell  Sarcoma).      {Original.) 


diseases  and  opthalmoplegia.  The  first  are:  titubation  or  staggering 
gait  and  incoordination  in  standing;  the  latter  is  bilateral  and  external. 
Nystagmus  is  also  a  frequent  symptom  (see  details  on  pages  213,  214,  215). 
Tumors  of  the  Basal  Ganglia  and  of  the  Corpus  Callosum. — The 
function  of  basal  ganglia  is  unsettled.     In  diseases  of  these  ganglia  the 


TUMORS    OF   THE   BRAIN  1 53 

symptoms,  present  nothing  pathognomonic.  There  are  also  cases  on 
record  in  which,  tumors  were  not  suspected  and  surprisingly  found  in 
this  region.  However,  in  view  of  the  close  proximity  of  the  internal 
capsule  (see  Anatomy),  pressure  symptoms  of  the  latter  will  frequently 
be  present,  namely  hemiplegia,  heminanaesthesia  and  hemianopsia, 
according  to  the  segment  of  the  capsule  involved  (see  Diseases  of  Basal 
Ganglia  and  Fig.  73). 

Tumors  of  Corpus  Callosum.  Symptoms. — The  general  symptoms 
observed  ordinarily  in  tumors  of  the  brain,  viz.  headache,  vomiting, 
vertigo,  optic  neuritis  and  convulsions,  are  only  exceptionally  met  with  in 
tumors  of  corpus  callosum.  Special  symptoms  :  Mental  apathy,  indiffer- 
ence are  very  early  manifestations.  The  diminution  of  activity  may 
progress  to  absolute  immobility.  Somnolence  is  another  frequent  symp- 
tom. Progressive  inability  of  concentrating  thoughts,  lack  of  atten- 
tion, increasing  depression,  progressive  diminution,  of  mental  faculties 
may  gradually  terminate  in  dementia.  Amnesia  may  be  observed. 
Confusion,  delirium  and  delusions,  with  hallucinations  have  been 
observed. 

Motor  Paralysis  and  Contractures. — Hemiplegia  is  the  most  frequent 
symtom,  but  what  is  particularly  characteristic  is  its  progressive  de- 
velopment. The  face  is  usually  spared.  Flaccidity  of  the  limbs  is  the 
most  frequent,  contractures  are  observed  in  the  minority  of  cases. 
Bilaterality  of  paralysis  is  less  frequent  than  unilaterality.  Monoplegia, 
paraplegia,  hemiplegia  on  one  side  and  monoplegia  on  the  opposite  side 
may  all  be  observed. 

Tremor,  Choreiform  movements  are  seen  sometimes.  Ataxia,  Dis- 
turbance, of  equilibrium  and  of  gait  have  been  observed  in  some  cases. 
The  tendon  reflexes  are  exaggerated  in  the  majority  of  cases.  The  toe 
phenomenon  is  occasionally  seen.  Sensations  are  rarely  involved.  Dis- 
turbances of  speech  are  frequent.  They  may  simulate  the  speech  of 
paretics;  dysarthria,  aphasia,  staccato  speech  have  all  been  observed. 
A  muttering  speech  or  speech  of  intonation  (Brissaud)  is  sometimes 
present;  the  patient  moves  his  lips,  but  does  not  make  sounds.  Apraxia 
is  a  very  serious  manifestation.  Its  relation  to  the  corpus  callosum  has 
been  established  by  Liepmann  (see  chapter  on  Apraxia,  page  138).  The 
sphincters  are  not  rarely  involved.  Cranial  nerves  are  usually  not  in- 
volved.    Occasionally  ocular  disturbances  are  observed. 

The  clinical  localizations  of  the  symptoms  are,  according  to  Schupter 
and  Duret,  as  follows: 

(1)  Anterior  portion  of  corpus  callosum:  psychic  disturbances, 
speech  symptoms  and  motor  disorders. 


154  TUMORS    OF   THE   BRAIN 

(2)  Middle  portion:  stupor,  titubation,  paretic  condition  of  the  ex- 
tremities. 

(3)  Posterior  portion:  disturbance  of  gait,  paresis  of  the  lower 
extremities,  cerebellar  symptoms. 

Course  and  Duration. — The  majority  of  writers  believe  that  the  on- 
set is  acute  and  the  disease  progresses  rapidly,  especially  the  mental 
symptoms. 

The  duration  is  usually  of  three  or  four  months.  There  are  however 
cases  of  longer  duration.  In  such  cases  a  careful  investigation  will 
almost  always  reveal  a  history  of  some  ill-defined  psychic  manifestations, 
disturbances  of  affectivity  and  of  character;  some  infectious  process  will 
become  the  exciting  cause  for  the  rapid  evolution  of  the  disease. 

Pathogenesis. — The  knowledge  of  apraxia  (page  138)  is  a  proof  of 
the  fact  that  the  corpus  callosum  accentuates  the  well-known  supremacy 
of  the  left  hemisphere  in  the  mechanism  of  thoughts.  The  left  hemi- 
sphere accumulates  certain  impressions  acquired  by  the  right  hemi- 
sphere. On  other  hand,  it  controls  the  acts  executed  by  the  right  hemi- 
sphere; the  latter  acts,  but  the  left  hemisphere  makes  it  act.  There 
is  a  sort  of  double  current  traveling  through  the  corpus  callosum,  viz. 
one  from  the  right  to  left,  the  other  from  the  left  to  right.  In  this  double 
function  one  finds  the  explanation  of  both  syndromes,  viz.  one  is  the 
mental  state  isolated  by  Raymond,  the  other  is  apraxia  emphasized  by 
Liepman. 

Tumors  of  the  Pituitary  Body. — In  diseases  of  the  hypophysis  cerebri 
two  conditions  may  be  observed:  one  is  acromegaly  and  gigantism 
(see  these  chapters),  the  other  is  adipositas  with  or  without  genital  hypo- 
plasia. Hyperpituitarism  is  probably  the  cause  of  the  former,  hypo- 
pituitarism of  the  latter.  The  clinical  symptoms  of  tumors  of  the  hypo- 
physis are,  besides  acromegaly  and  adipositas,  also  the  following:  bitemporal 
hemianopsia  caused  by  pressure  on  the  chiasma,  headache,  somnolence 
occurring  paroxysmally,  inability  to  concentrate  thoughts  and  impair- 
ment of  memory.  Frequently,  involvement  of  the  third  and  sixth 
nerves  is  observed.  Exophthalmos  may  occur  in  advanced  stages  when 
the  tumor  after  destroying  the  sella:  turcica  presses  on  the  orbital  cavity. 
Loss  of  smell  may  be  present.  The  latter  two  symptoms  occurred  in 
one  of  my  cases  (/.  Am.  Med.  Ass.,  191 2).  Hemiplegia  or  generalized 
or  focal  convulsions  may  also  occur. 

The  adiposity  together  with  sexual  infantilism  constitutes  the  so-called 
Frohlich's  syndrome  or  syndrome  adiposo-genital.  The  patient  thus 
affected  keeps  on  laying  on  fat  in  spite  of  the  exceedingly  small  amounts  of 
food  which  he  may  consume.     The  genitalia  are  atrophied  (testicles  and 


TUMORS    OF    THE   BRAIN 


155 


ovaries).  Glycosuria  and  excessive  sweating  are  not  infrequently  ob- 
served. They  may  be  the  result  of  the  disturbed  function  of  the  hy- 
pophysis (Fig.  74). 

Course.     Termination.     Prognosis. — The   malignant  tumors   (carci- 
noma, sarcoma,  etc.)  run  a  rapid  course.     Solitary  tubercle,  glioma  or  osteo- 


Fig.  74. — Tumor  of  Hypophysis,  Showing  Two  Lobes.     Crura  and  Pons  are  Deformed 

(Adipositas  Cerebralis.)     {Original.) 

sarcoma  develop  much  more  slowly  than  a  sarcoma  or  carcinoma.  The 
latter  is  fatal  in  two  to  three  years  and  in  children  in  two  months.  A 
cavernous  angioma  may  persist  for  years.  A  tubercle  may  become  station- 
ary or  undergo  ossification  or  calcification.  Young  individuals  bear  tumors 
at  the  base  of  the  brain  better  than  adults  owing  to  superior  elasticity 


156  TUMORS    OF    THE  BRAIN 

and  yielding  capacity  of  bones  and  sutures  of  the  skull.  The  develop- 
ment of  cerebral  tumors  is  as  a  rule  inevitably  progressive  and  death  is 
the  common  termination.  In  some  cases  the  evolution  of  a  tumor  may 
be  very  insidious  and  the  symptoms  very  mild,  so  that  diagnosis  of  hys- 
teria, migraine,  psychoneurosis,  eye  strain,  dyspepsia  is  made.  This 
may  go  on  for  years  until  an  apoplectiform  or  an  epileptiform  attack 
occurs.  Then  the  symptoms  become  pronounced  and  advance  rapidly. 
Such  an  occurrence  is  possible  when  a  tumor  is  located  in  the  so-called 
"silent"  area  of  the  brain,  under  which  term  is  understood  any  portion 
of  the  brain  except  the  well-known  regions,  the  function  of  which  is  well 
studied.  In  these  cases  a  tumor  may  give  rise  to  pressure  symptoms 
alone  but  cannot  be  localized  even  if  its  presence  is  suspected.  Sudden 
death  may  take  place  in  the  course  of  cerebral  tumors.  On  the  other 
hand  arrest  of  development  of  symptoms  is  possible  for  a  more  or  less 
prolonged  period  until  an  accident,  an  injury  or  some  other  cause  will 
give  an  impetus  to  the  silent  tumor,  which  then  begins  to  progress  rapidly. 
Gummata  when  treated  energetically  run  a  different  course:  under  this 
condition  the  symptoms  may  be  greatly  relieved. 

Generally  speaking,  cerebral  growths  belong  to  the  most  serious 
affections.  In  a  small  group  of  cases  in  which  strictly  localized  symp- 
toms indicate  the  exact  seat  of  the  neoplasm,  an  operation  may  improve 
the  condition  considerably  and  even  remove  the  symptoms.  But  even 
then  recurrences  are  possible.  The  most  favorable  prognosis  present 
gummata.  Arrest  of  symptoms  is  observable  in  tubercular  tumors, 
and  according  to  some  these  neoplasms  are  capable  of  spontaneous 
retrogression.  Tumors  are  liable  to  become  softened,  cheesy  in  center 
and  reduced  in  size.  They  are  likely  to  become  encapsulated.  In 
some  cases  spontaneous  hemorrhages  occur  in  the  tumor  thus  check- 
ing its  growth.  The  outlook  in  superficially  situated  tumors,  such 
as  in  the  meninges  and  cortex,  is  more  favorable  than  in  deep-seated 
tumors. 

Diagnosis. — Individual  symptoms  of  brain  tumors  may  be  encountered 
in  other  affections,  so  that  in  order  to  make  a  diagnosis,  the  latter  must 
be  excluded. 

Headache  and  vomiting  occur  in  migraine,  but  the  cerebral  character 
of  vomiting  (see  above)  and  the  persistent  character  of  the  pain  in  the 
head  will  exclude  the  latter  affection  (see  Migraine). 

Abscess  of  the  brain  will  be  distinguished  from  tumor  by  rapid  de- 
velopment of  symptoms;  the  latter  are  very  pronounced  in  abscess  and 
gradually  progressing  in  tumors.  Optic  neuritis  is  very  rare  in  abscess, 
common  in  tumor.     The  etiological  factors,  as  suppurative  disease  of 


TUMORS    OF    THE   BRAIN  157 

the  ears  or  of  neighboring  tissues  or  cavities,  are  common  in  abscess 
(see  also  chapter  on  Abscess). 

Chronic  meningitis  of  alcoholic  or  syphilitic  nature  may  be  confounded 
with  tumors.  The  identity  of  the  optic  nerve  condition  in  both  cases 
makes  the  diagnosis  very  difficult.  This  is  particularly  applicable  to 
localized  meningitis.  When  the  meningitis  is  tubercular,  the  differen- 
tiation is  easier,  as  optic  neuritis  is  less  frequent  and  the  headache  more 
severe  than  in  brain  tumors. 

An  error  may  be  committed  in  making  a  diagnosis  of  hydrocephalus. 
The  congenital  character  of  the  latter,  the  form  of  the  head,  the  spastic 
palsy  of  two  symmetrical  extremities  without  convulsions  will  help  to 
eliminate  a  cerebral  neoplasm. 

In  order  to  differentiate  from  cerebral  hemorrhage  or  softening, 
the  etiological  factors  must  be  taken  into  consideration  (see  Apoplexy). 
Moreover  absence  of  optic  nerve  changes  is  the  rule  in  apoplexy. 

The  diagnosis  between  frontal  tumor  and  paresis  is  sometimes  difficult. 
Cases  have  been  reported  in  which  the  mental  phenomena  of  paresis 
were  found  in  frontal  tumors.  In  such  cases  the  cerebro-spinal  fluid  may 
decide  the  diagnosis:  lymphocytosis  and  increase  of  albumen  are  present 
in  paresis,  but  very  rarely  in  tumors. 

In  some  tumors  of  the  brain  epilepsy  may  be  the  only  symptom  for  a 
long  time.  The  diagnosis  is  then  very  difficult.  It  is  only  when  optic 
neuritis  and  headache  make  their  appearance  that  the  distinction  can  be 
made. 

The  most  important  role  in  cerebral  tumors  play  the  localized  symp- 
toms, when  they  develop  gradually.  It  should  not  be  forgotten  that 
certain  tumors  may  remain  latent  for  a  long  time  and  be  unaccompanied 
by  the  characteristic  symptoms  until  a  hemorrhage  occurs  into  them. 
Then  the  paralytic  or  other  focal  symptoms  will  determine  the  diagnosis. 

As  to  the  seat  of  the  tumor,  the  localizing  symptoms  already  described 
for  each  individual  area  of  the  cortex,  also  for  differentiation  of  cortical 
and  subcortical  growths,  are  to  be  taken  into  consideration. 

The  nature  of  the  tumor  can  be  determined  with  great  difficulty. 
The  patient's  personal  and  family  history  should  be  investigated.  The 
concomitant  existence  of  cancerous,  sarcomatous  or  other  growths  in 
other  organs  of  the  body,  the  existence  of  phthisis,  a  history  of  acquired 
syphilis,  the  positive  or  negative  Wasserman  reaction,  all  these  informa- 
tions are  important  factors  in  deciding  the  question  of  the  variety  of  a 
given  tumor.  Improvement  observed  during  the  administration  of  cer- 
tain drugs  is  valuable  only  to  some  extent  in  determining  the  nature  of 
the  tumor.     Mercury  and  iodides,  for  example,  have  a  beneficial  effect 


158  TUMORS    OF   THE  BRAIN 

in  syphilitic  growths,  but  this  is  not  absolute,  as  improvement  has  been 
also  observed  with  the  same  drugs  in  gliomata  or  tuberculomata.  The 
seat  of  the  tumor  has  some  bearing  upon  the  determination  of  its  nature. 
Thus  tumors  on  the  surface  of  the  brain  are  frequently  syphilitic,  tuber- 
culous or  sarcomatous.  In  the  subcortical  tissue,  mostly  gliomatous. 
In  the  cerebellum,  tuberculous.  The  duration  is  equally  important: 
tuberculous  and  gliomatous  tumors  are  long;  syphilitic  when  not  treated, 
also  carcinomatous  growths  run  a  rapid  course. 

In  recent  years  X-rays  have  been  applied  for  diagnostic  purposes 
in  studies  of  localization  of  brain  tumors.  Although  the  results  are  not 
absolutely  satisfactory  in  every  case,   they  are  nevertheless  promising. 

The  determination  of  the  nature  of  cerebral  tumors  presents  particular 
difficulties  in  parasitic  cysts  of  the  brain  (echinococcus).  The  affection  is 
comparatively  rare.  According  to  the  statistics  of  Cranwell  and  H. 
Vegas  of  Buenos- Ayres  among  1.696  parasitic  cysts  of  various  organ 
only  36  were  found  in  the  brain,  of  which  27  were  in  children.  The 
diagnosis  of  cerebral  echinococcus  is  extremely  difficult,  as  there  are  no 
pathognomonic  manifestations.  Parvu  and  Laubry  {Bull,  de  Soc.  Med:, 
d.  hop.,  1908)  have  observed  that  if  the  blood  serum  of  individuals  having 
the  echinococcus  in  any  other  organ  contain  specific  antibodies,  the  latter 
are  absent  in  the  cerebro-spinal  fluid,  but  they  will  be  present  if  the 
central  nervous  system  is  affected.  Recently  (Press.  Med.,  191 1), 
Legry,  Parvu  and  Baumgartner  reported  new  proofs  of  the  above  obser- 
vation. A  positive  deviation  of  complement  in  the  cerebro-spinal 
fluid  and  negative  in  serum  is  pathognomonic  of  a  parasitic  cyst  in  the 
brain  when  a  tumor  is  suspected  and  when  no  other  cause  could  be 
accounted  for. 

Etiology.- — The  causes  of  brain  tumors  are,  generally  speaking,  the 
same  as  in  tumors  of  any  other  organ.  Trauma  has  been  considered  for 
a  long  time  as  an  exciting  cause  for  development  of  intracranial  growths. 
The  symptoms  of  tumor  may  begin  to  appear  shortly  after  the  trauma 
or  even  years  later.  It  is  reasonable  to  presume  that  an  injury  plays  the 
role  of  a  provoking  agent  for  a  rapid  development  of  a  tumor  which  existed 
before  the  injury.  It  has  been  also  proven  that  some  tumors  are  of  a 
congenital  origin,  as  coexistence  of  tumors  with  cerebral  malformations 
has  been  observed.  There  are  good  reasons  to  believe  that  glioma, 
which  is  met  with  mainly  in  young  people,  is  of  fcetal  origin.  Tubercular 
tumors  are  usually  secondary  to  pulmonary  tubercular  affections. 
Syphilitic  growths  are  chiefly  acquired  after  an  initial  specific  infection. 

Treatment. — It  should  be  the  rule  in  every  case  of  brain  tumor  to 
give  the  patient  the  specific  treatment  for  a  period  of  several  weeks. 


TUMORS    OF    THE   BRAIN 


159 


If  the  tumor  is  of  specific  character  the  patient  may  derive  great  benefit. 
In  two  of  my  very  recent  cases  there  was  a  remarkable  retrogression  of 
the  headache,  of  vertigo  and  of  the  choked  disc  under  the  influence  of 
large  doses  of  potassium  iodide  (150  gr.  t.i.d.)  associated  with  mercurial 


Fig.  75. — Situation  of  a  Tumor,  shown  from  Frazer's  Guide  to  Cerebral  Operations. 
The  figure  shows  the  relation  of  the  convolutions  to  the  head. 

inunctions  (5j  twice  daily).  On  the  other  hand,  some  tumors,  gliomata, 
for  example,  sometimes  improve  considerably  under  mercurials  and 
iodides.     The   so-called   expectant    treatment,  viz.  the  treatment   with 


l6o  TUMORS    OF    THE  BRAIN 

antisyphilitic  drugs  for  a  period  of  weeks  in  the  light  of  accumulated 
experience  should  be  abandoned  in  spite  of  amelioration  of  some  of  the 
symptoms.  Not  only  is  it  useless  as  far  as  the  removal  of  the  growth  is 
concerned,  but  it  may  so  considerable  damage  because  waiting  means  an 
increasing  opportunity  for  the  progressive  growth  to  destroy  the  cerebral 
tissue.  Complete  recovery  or  great  amelioration  of  symptoms  can  be 
expected  only  from  surgical  intervention.  The  latter  consists  of  the 
following  procedures:  (i)  Lumbar  puncture,  (2)  puncture  of  the  corpus 
callosum,  (3)  palliative  operation  and  (4)  radical  operation  for  removal  of 
the  tumor. 

Lumbar  puncture  has  given  to  some  satisfactory  and  to  others  unsatis- 
factory results.  Sicard  has  recently  (Presse.  Med.,  1908)  formulated  the 
following  rules  which  if  followed  strictly  will  give  favorable  results.  For 
48  hours  the  patient  must  be  be  kept  in  bed  before  the  lumbar  puncture  is 
made;  the  head  must  not  to  raised  during  that  time.  The  puncture  is 
made  in  Trendelenburg's  position  (head  lower  than  body),  the  patient 
placed  on  his  side.  The  same  position  is  to  be  kept  for  the  following 
12  or  24  hours,  then  the  patient  is  placed  horizontally  for  48  hours. 
Lumbar  puncture  should  never  be  made  when  headache,  vertigo,  etc.,  are 
aggravated  by  a  horizontal  position.  I  have  obtained  great  amelioration 
of  the  distressing  symptoms  of  cerebral  growths  in  a  number  of  cases  by 
means  of  lumbar  puncture.  In  three  cases  of  tumor  in  the  cerebello- 
pontine angle  a  similar  procedure  aided  me  in  improving  the  patient's 
general  condition  and  thus  prepare  him  for  the  major  operation.  I  have 
never  met  with  untoward  results. 

Puncture  of  the  corpus  callosum  has  been  advocated  particularly  by 
Anton  and  Bramann  {Munch,  mediz.  Wochenschr.,  1908).  From  an 
experience  of  40  cases  they  draw  the  conclusion  that  this  procedure  estab- 
lishes a  free  communication  between  all  the  ventricles  and  the  subdural 
space.  It  relieves  promptly  the  headache,  vertigo  and  vomiting,  also  the 
hyperemia  and  stasis  of  the  papillae.  By  preventing  thus  rapid  blindness, 
it  helps  gaining  time  so  as  to  prepare  the  patient  for  an  eventual  radical 
operation.  During  the  puncture  of  the  corpus  callosum  the  ventricles 
should  be  probed  so  that  any  abnormal  resistance  in  them  should  be 
destroyed. 

Palliative  operation,  undertaken  in  cases  of  inoperable  tumor,  has  for 
object  cerebral  decompression.  It  consists  of  removal  of  a  portion  of 
bone  in  the  temporal  or  occipital  areas  and  of  opening  the  dura.  Resto- 
ration or  preservation  of  sight,  diminution  of  optic  neuritis,  relief  from, 
agonizing  headache  and  vomiting,  have  all  been  observed  after  cerebral 
decompression.     In  sub-tentorial  tumors  the  operation  is  performed  in  the 


TUMORS    OF    THE   BRAIN  l6l 

sub-occipital  region,  in  all  other  cases  the  temporal  region  on  one  and  later 
on  the  other  side  is  the  seat  of  choice. 

In  inoperable  tumors,  viz.  when  the  neoplasm  is  so  situated  that  it  can- 
not be  removed,  or  when  localization  is  impossible  or  when  the  gravity  of 
the  condition  is  such  that  delay  means  loss  of  sight,  decompression  is  urgent 
and  at  the  earliest  possible  moment.  While  blindness  is  not  prevented 
in  every  operated  case,  nevertheless  the  positive  results  obtained  in  some  are 
sufficient  to  warrant  the  necessity  for  decompression.  In  Cushing's  sta- 
tistics of  130  cases,  59  had  increased  vision,  65  remained  the  same  and 
16  had  decreased  vision.  Cushing's  new  idea  of  performing  decompression 
on  the  side  opposite  to  that  occupied  by  the  tumor  seems  to  meet  with 
favor. 

That  operative  procedures  may  give  excellent  results  can  be  also  seen 
from  the  instructive  statistics  of  Allen  Starr,  who  says  that  out  of  361  op- 
erated cerebral  tumors  154  recovered.  Oppenheim's  statistics  show  11.4 
per  cent,  recoveries.  Operations  should  be  performed  as  early  as  possible, 
as  the  smaller  the  tumor  the  less  damage  will  be  done  to  the  cerebral  tissue. 
As  to  the  variety  of  surgical  procedures,  osteoplastic  operations  have  proven 
to  be  of  more  value  and  followed  by  better  results  than  trephining. 
The  operation  should  be  performed  as  near  as  possible  to  the  lesion, 
so  that  if  subsequently  it  be  decided  to  attempt  the  radical  operation, 
there  will  be  less  damage  to  the  brain  tissue.  As  to  the  seat  of  the  tumor, 
this  is  to  be  determined  by  a  close  neurological  examination  as  to  motor 
and  sensory  symptoms,  reflexes,  localized  convulsive  seizures,  etc. 

Horsley  recommends  in  decompressive  operations,  irrigation  of  the  sub- 
dural space  with  sublimate  solutions  (1:1000).  This  procedure  has 
given  him  good  results. 

A  few  words  in  regard  to  the  medical  treatment.  The  latter  is  only 
palliative.  Besides  specific  drugs,  which  may  give  favorable  results  in 
some  cases,  drugs  must  be  given  for  alleviating  pain,  vomiting,  vertigo  or 
insomnia.  Any  of  the  coal-tar  products  (salicylates,  phenacetin,  anti- 
pyrin,  aspirin,  etc.),  must  be  given  before  an  operation  is  decided  upon. 
Bromides,  hyoscine,  codein  may  relieve  the  vomiting,  the  pain,  the  vertigo 
and  the  convulsions.  In  some  cases  ergot  is  of  great  benefit,  when 
other  means  fail.  Veronal  should  be  given  for  insomnia.  The  diet  must 
be  light  and  given  in  small  quantity;  the  bowels  should  be  kept  freely 
open.  In  tubercular  cases  when  relief  is  obtained  from  the  distressing 
symptoms  by  the  above  means,  cod-liver  oil,  iron  and  arsenic  should  be 
administered. 


CHAPTER  X 

HYDROCEPHALUS 

Hydrocephalus  is  characterized  by  an  excessive  accumulation  of 
fluid  in  the  cranial  cavity.  In  the  majority  of  cases  it  occupies  the 
ventricles  (internal  hydrocephalus),  but  it  may  also  accumulate  in  the 
subdural  space  (external  hydrocephalus) . 

VARIETIES  OF  HYDROCEPHALUS 

I.  (a)  Chronic  Congenital. — This  form  is  associated  with  malfor- 
mations of  the  brain.  The  hemispheres  of  the  cerebrum  and  cerebel- 
lum may  be  entirely  wanting  (anencephaly) ,  may  present  absence  of 
certain  lobes  or  of  other  portions  (as  corpus  callosum,  etc.),  or  they  may 
be  reduced  in  size  (microcephaly  or  microgyria).  (See  chapter  on  Mal- 
formations.) With  the  congenital  hydrocephalus  are  frequently  asso- 
ciated symptoms  of  arrested  development  of  various  tissues  or  organs  of 
the  body  (spina  bifida,  ectopia  of  organs,  flat-foot,  hare-lip,  etc.). 

(b)  Chronic  Symptomatic  Hydrocephalus. — This  form  is  secondary 
to  other  morbid  processes.  Cerebral  (especially  at  the  base)  and  mostly 
cerebellar  tumors,  through  compression  of  the  sinuses  of  the  dura  and  of 
Galen  veins,  will  interfere  with  the  return  circulation  and  produce  ven- 
tricular dropsy.  Quincke  called  attention  to  excessive  exudation  of  cere- 
brospinal fluid  in  the  ventricles  caused  by  serous  meningitis.  Finally 
hydrocephalus  may  be  symptomatic  of  phlebitis  of  the  sinuses. 

II.  Acute. — It  is  a  rare  affection.  It  is  due  to  an  infectious  or  toxic 
condition  (gastro-intestinal  diseases).  The  effusion  is  more  frequently 
in  the  ventricles  than  in  the  subdural  space.  In  the  first  case  the  inflam- 
mation affects  the  choroid  plexus  and  the  ependyma  of  the  ventricles; 
in  the  second  case,  the  pia-mater,  and  it  is  therefore  the  result  of  an  acute 
serous  meningitis. 

III.  External  hydrocephalus  is  by  far  less  frequent  than  internal. 
It  is  practically  a  subdural  cyst  and  the  result  of  a  localized  meningitis. 

Pathology. — In  the  ventricular  form  the  following  condition  is  found. 
Among  all  the  ventricles  the  lateral  ones  are  chiefly  affected.  The 
amount  of  fluid  is  variable;  two  quarts  is  not  a  very  rare  occurrence. 
The  fluid  is  clear,  with  a  specific  gravity  of  1005  to  1010.     It  contains 

162 


HYDROCEPHALUS 


163 


a  small  quantity  of  albumen  and  a  noticeable  amount  of  chlorides  (more 
than  in  the  plasma  of  blood).  The  distention  of  the  ventricles  may  be 
symmetrical  on  both  sides  or  may  be  more  pronounced  on  one  side  than 
on  the  other.  When  the  orifices  of  Monro  are  dilated,  the  third  ventricle 
and  the  aqueduct  of  Sylvius  are  distended,  the  dilatation  will  reach  the 
fourth  ventricle. 

The  lining  membrance  of  the  ventricles  and  the  choroid  plexuses 
are  thickened.  The  cerebral  tissue,  being  compressed,  is  reduced  in 
thickness.  The  convolutions  are  flattened  and  the  fissures  disappear. 
In  hydrocephalus  occurring  very  early  in  life,  myelinization  of  the  nerve 
fibers  is  arrested.  The  pyramidal  fibers  through  their  entire  course 
present  in  this  case  an  atrophic  condition. 
In  hydrocephalus  appearing  later  in  life  the 
pyramidal  tract  is  in  a  state  of  secondary 
degeneration.  The  cranium  is  enlarged  and 
globulous.  The  fontanelles  are  large,  the 
sutures  are  separated. 

Symptoms. — The  most  conspicuous  ini- 
tial symptom  is  the  gradual  increase  of  the 
size  of  the  head.  In  some  cases,  however, 
spasticity  of  the  extremities  or  convulsions 
may  precede  the  abnormal  development  of 
the  head. 

The  head  is  usually  enlarged  in  its  trans- 
verse diameter,  the  sutures  are  separated, 

the  forehead  is  unusually  prominent.  The  facies  is  striking:  it  is  pale 
and  thin,  the  subcutaneous  veins  are  prominent,  the  eyes  are  protrud- 
ing and  directed  downward.  The  patient  is  unable  to  hold  the  head 
erect,  as  the  latter  has  a  tendency  to  fall  back.  The  hair  on  the  head  is 
sparse. 

Nervous  symptoms  are  mainly  motor.  Convulsions  appear  early; 
they  are  due  to  pressure  upon  the  cortex  produced  by  the  dilated  ven- 
tricles. The  contractures  of  the  extremities  and  weakness,  which  are 
often  present,  are  incomplete.  The  tendon  reflexes  are  increased  and 
the  toe  phenomenon  is  present.     The  gait  is  difficult  and  uncertain. 

General  sensibility  is  rarely  affected,  but  among  the  special  senses 
the  vision  is  very  frequently  and  quite  early  affected  in  hydrocephalus 
because  of  pressure  exerted  by  the  dilated  third  ventricle  upon  the 
chiasma.  Optic  neuritis  followed  by  optic  atrophy  is  a  common  occur- 
rence. Strabismus  is  also  commonly  observed.  Mental  development 
is  frequently  arrested.     There  may  be  idiocy  or  only  feeble-mindedness. 


= 

w^ 

D 

W; 

•''".•■;  a?";^p 

f/sfslH 

•'.'.|#v;- 

'■■■•■■■ir'va 

Fig.  76. — Hydrocephalus. 
(Oppenheim.) 


164  HYDROCEPHALUS 

All  the  faculties  are  retarded  in  early  hydrocephalus;  thus  the  speech  is 
acquired  late. 

As  the  disease  is  usually  progressive,  the  intracranial  tension  and 
pressure  continue  to  increase.  Headache  is  therefore  a  frequent  symp- 
tom and  the  suffering  sounds  made  by  the  patient  are  so  characteristic 
that  they  received  a  special  name,  "hydrocephalic  cry."  It  is  a  sort  of  a 
shallow  scream  which  does  not  resemble  any  sound  made  by  patients  in 
other  painful  affections.  In  adults  the  fontanelles  do  not  separate  and 
therefore  the  enlargement  of  the  head  is  not  so  conspicious  as  in  children. 
The  condition  may  then  be  confounded  with  cerebral  tumor.  Indeed 
the  general  symptoms  of  the  latter  are  all  present,  viz.  headache,  vertigo, 
optic  neuritis,  convulsions.  The  diagnosis  is  difficult.  However,  early  loss 
of  upward  movement  of  the  eyes,  early  impairment  of  light  reflex,  progress- 
ive bilateral  rigidity,  are  all  almost  pathognomonic  of  hydrocephalus 
in  adults.  The  reason  of  the  ocular  phenomena  is  to  be  found  in  the 
downward  pressure  exerted  by  the  distended  third  ventricle  on  the  nucleus 
of  the  third  nerve. 

Prognosis. — The  outlook  is  very  serious.  Death  usually  occurs  at 
an  early  age  and  most  frequently  from  some  intercurrent  disease.  There 
are,  however,  cases  which  may  recover,  but  they  are  very  rare.  Some 
writers  reported  a  few  cases  in  which  the  hydrocephalic  fluid  found  a 
passage  through  the  nasal  fossae  and  through  the  ears  after  a  fracture  of 
the  skull.  That  hydrocephalus  may  become  arrested  in  its  development 
is  a  well  known  fact:  the  sutures  become  then  ossified  and  the  head 
ceases  to  grow  large;  the  individual  may  live  long. 

Diagnosis.  Chronic  Hydrocephalus. — As  the  large  size  of  the  head 
is  the  most  prominent  symptom  in  hydrocephalus,  the  latter  may  be 
confounded  with  rickets,  in  which  the  head  may  be  also  voluminous. 
In  the  latter  affections  there  is  usually  no  intellectual  disturbance;  the 
fontanelles  are  not  enlarged,  the  cranium  is  not  uniformly  enlarged; 
finally  the  usual  stigmata  of  rickets  (deformities  of  limbs,  etc.)  will  render 
the  diagnosis  comparatively  easy. 

Acute  Hydrocephalus. — Acute  Cerebral  Affections  of  early  infancy 
may  simulate  hydrocephalus.  It  is  therefore  important  in  such  cases 
to  examine  frequently  the  state  of  the  fontanelles  and  of  the  sutures. 
Tubercular  meningitis  in  infants  may  also  resemble  hydrocephalus.  In 
making  a  diagnosis  it  should  be  remembered  that  tubercular  meningitis 
occurs  habitually  in  children  that  are  weak  or  directly  tubercular.  More- 
over the  cerebro-spinal  fluid  in  tubercular  meningitis  presents  some 
special  features,  viz.  increase  of  albumen  and  especially  lymphocytosis 
and  the  tubercle  bacilli.     A  lumbar  puncture  will  therefore  decide  the 


HYDROCEPHALUS  I 65 

differential  diagnosis.  The  greatest  difficulty  lies  in  differentiating  in- 
ternal from  external  hydrocephalus.  However  the  enlargement  of  the 
head  in  ventricular  forms  is  more  rapid  than  in  external  forms.  Besides, 
at  the  beginning  of  external  hydrocephalus  the  symptoms  are  those  of 
hemorrhagic  meningitis  or  pachymeningitis,  and  the  latter  affections 
lead  rapidly  to  death. 

Etiology. — In  congenital  forms  of  hydrocephalus  heredity  plays  a 
great  role.  There  is  almost  always  a  history  of  some  nervous  or  mental 
disorder  or  else  some  constitutional  disease  in  the  family,  as  tuberculosis, 
alcoholism  and  especially  syphilis.  Consanguinity  of  the  parents  has 
also  been  reported  as  an  etiological  factor.  Fournier  and  Hutchinson 
call  special  attention  to  the  effect  of  syphilis  of  the  parents  on  the  pro- 
duction of  hydrocephalus  in  the  offspring.  During  pregnancy  physical 
injuries  to  the  foetus  may  lead  to  pathological  changes  and  cause  hydro- 
cephalus, as,  for  example,  compression,  violence,  etc. 

Hydrocephalus,  which  makes  its  appearance  in  infancy,  develops 
most  frequently  in  the  course  of  gastro -intestinal  diseases.  The  mech- 
anism of  formation  of  the  affection  is  various:  it  may  be  thrombosis 
of  the  venous  sinuses  or  inflammation  of  the  ventricular  lining  membrane 
and  of  choroid  plexuses,  with  the  result  of  abundant  serous  exudation. 
Rickets  is  not  infrequently  associated  with  hydrocephalus  and  therefore 
may  be  considered  as  a  cause  of  the  latter.  Trauma  of  the  head  has  also 
been  mentioned  as  a  cause  of  acquired  hydrocephalus  (Plehn,  Quincke, 
Finkelnburg  and  Nonne).  The  etiology  of  the  symptomatic  forms  of 
hydrocephalus  depends  upon  the  accompanying  cerebral  affection  (see . 
above). 

Treatment.  Chronic  Hydrocephalus.— In  view  of  the  great  role 
which  syphilis  plays  in  the  etiology  of  this  affection,  a  vigorous  specific 
treatment  must  be  instituted  as  early  as  possible.  While  a  complete 
cure  should  not  be  expected,  amelioration  of  symptoms  may  be  obtained. 
Hygienic  measures  aided  by  a  proper  diet  will  improve  the  general  health 
of  the  patient.  In  acute  hydrocephalus  the  treatment  must  be  directed 
against  the  cause.  As  gastro-intestinal  diseases  are  a  frequent  cause, 
removal  of  the  latter  must  be  the  principal  aim:  an  appropriate  diet  with 
intestinal  antiseptics,  local  applications,  etc.,  must  be  administered. 

Surgical  intervention,  still  advocated  by  some,  is  a  serious  procedure. 
It  consists  either  of  puncturing  the  cerebral  ventricles  or  of  a  lumbar 
puncture.  Both  methods  are  uncertain:  observation  shows  that  the 
patients  either  die  from  sudden  decompression  of  the  brain,  when  too 
much  fluid  is  withdrawn,  or  from  secondary  inflammatory  complications 
following  the  puncture.     Moreover,  the  fluid  reforms  immediately  after 


1 66  HYDROCEPHALUS 

the  puncture;  repeated  punctures  will  therefore  be  necessary.  On  the 
other  hand  some  very  competent  authors  report  favorable  results  from 
both  procedures.  Anton  and  Bramann  (see  Treatment  of  Tumors 
of  Brain)  obtained  great  amelioration  of  the  general  symptoms,  viz. 
headache,  vertigo,  vomiting  also  of  ocular  disturbances  by  puncturing  the 
corpus  callosum  and  probing  the  lateral  ventricles.  Krause  (Zentralbl. 
j.  Chir.,  1908)  drained  directly  the  cavity  of  the  ventricle  into  the  sub- 
cutaneous tissue  of  the  scalp  by  means  of  a  silver  tube  inserted  through 
the  cortex  into  the  ventricle  and  held  in  place.  In  some  of  his  patients 
the  tube  was  held  in  place  for  several  months.  Some  favorable  results 
were  obtained.  Equally  good  results  were  reported  by  Finkelnburg 
(Miinch.  mediz.  Wchnschr.,  1910)  who  speaks  of  puncturing  the  motor 
area  at  a  depth  of  5  cm. 

Lumbar  puncture  may  also  improve  the  general  manifestations  of 
hydrocephalus  in  a  limited  number  of  cases. 


CHAPTER  XI 

DISEASES  OF  THE  BASAL  GANGLIA  (OPTIC  THALAMUS. 
CORPORA.  STRIATA.    CORPORA  QUADRIGEMINA) 

I.  THE  SYNDROME  OF  THALAMUS  OPTICUS 

It  was  first  brought  out  in  1903  by  Dejerine  and  Egger. 

Pathological  Physiology. — The  thalamic  syndrome  is  the  result  of  a 
lesion  in  the  external  and  internal  nuclei,  or  in  the  pulvinar  of  the  thala- 
mus; also  in  the  fibers  of  the  posterior  portion  of  the  internal  capsule. 
The  thalamus  being  essentially  a  sensory  organ  connecting  the  periphery 
with  the  cortical  centers  by  means  of  cortico- thalamic  and  thalamo-cortical 
fibers,  a  lesion  of  it  will  naturally  give  place  mainly  to  hemianaesthesia. 
The  latter  explains  the  hemiataxia  which  is  so  frequently  found  asso- 
ciated. The  motor  symptoms  find  their  explanation  in  the  involvement 
of  the  capsular  fibers. 

Symptoms. — They  are:  hemianaesthesia,  hemiataxia,  hemiplegia, 
choreiform  movements,  athetosis,  pain  on  the  anaesthetic  side.  Some- 
times mimicry  is  affected. 

Hemianaesthesia. — It  is  pronounced  for  deep  sensations  (muscular, 
tendinous,  articular  and  osseous)  and  slight  for  superficial  sensations 
(touch,  pain  and  temperature). 

The  muscular  sense,  position  of  the  limbs,  the  sense  of  active  and 
passive  movements,  of  resistance,  of  force,  of  weight  are  all  completely 
abolished. 

Hemiataxia.— It  is  slight  and  limited,  but  it  does  not  interfere  to  a 
great  extent  with  locomotion. 

Hemiplegia. — It  is  slight.  It  is  not  a  real  paralysis  but  only  a  slight 
diminution  of  power.  Contracture  does  not  develop.  It  has  a  tendency 
to  improve.  The  knee-jerks  are  usually  exaggerated.  Meynert,  Schiff, 
Clarke  and  a  few  others  observed  in  lesions  of  the  thalamus:  flexion  of 
the  arm  on  the  opposite  side,  extension  of  the  arm  on  the  same  side  and 
head  and  eyes  turned  to  the  opposite  side  of  the  lesion. 

Choreiform  Movements  and  Athetosis  are  not  marked.  They  are 
brought  out  especially  on  voluntary  movements.  Their  pathogenesis  is 
not  yet  definitely  established.  However  athetosis  appears  to  occur  not 
infrequently  in  lesions  of  the  thalamus. 

Pain  on  the  anaesthetic  side  is  either  spontaneous  or  brought  on  by  the 

167 


105  DISEASES    OF   THE   BASAL   GANGLIA 

least  pressure.  There  may  be  distinct  paroxysms  of  shooting  pain. 
Instead  of  pain  there  may  be  numbness,  burning  or  tingling. 

In  addition  to  the  above  symptoms  there  are  sometimes  hemianopsia, 
disturbances  of  the  bladder  and  trophic  disorders.  The  hemianopsia  is 
of  the  homonymous  type.  It  is  probably  due  to  the  involvement  of  the 
fibers  of  the  optic  tract  which  originate  in  the  pulvinar. 

In  a  few  cases  of  lesions  of  the  thalamus  the  following  phenomenon 
was  observed:  on  voluntary  movements  the  muscles  of  the  face  contract 
equally  on  both  sides,  but  in  the  act  of  laughing  or  crying  (emotional  act) 
the  motion  of  the  affected  side  is  considerably  diminished  or  else  abolished. 

Course. — The  onset  is  usually  sudden.  There  is  a  loss  of  consciousness 
preceded  by  some  vertigo.  The  motor  disturbances  improve  rapidly, 
while  the  sensory  persist  and  even  increase. 

Diagnosis. — A  marked  hemiansesthesia  associated  with  a  mild  hemi- 
plegia is  the  essential  feature  of  thalamic  syndrome.  However  there  are 
other  portions  of  the  central  nervous  system  which,  being  diseased,  may 
present  the  above  symptomatology,  but  in  such  cases  there  are  usually 
additional  manifestations  which  will  aid  in  making  a  diagnosis  (see 
Roussy's  thesis). 

II.  CORPUS  STRIATUM 

Lesions  confined  strictly  to  the  gray  matter  of  striate  bodies  are  very 
rare.  However  there  are  a  few  cases  on  record,  in  which  the  adjacent 
tissue  was  not  involved.  They  are,  exclusively,  hemorrhages.  The  symp- 
tom-group of  such  an  affection  has  been  recently  established  with  some 
precision  by  S.  A.  K.  Wilson  (Brain,  V.  XXXIV).  He  describes  the  affec- 
tion under  the  name  of  "progressive  lenticular  degeneration,"  which  can 
be  considered  as  the  syndrome  of  the  corpus  striatum.  Pathologically 
there  is  total  integrity  of  the  internal  capsule  and  pyramidal  paths, 
but  there  is  a  bilateral  softening  and  degeneration  of  the  lenticular  nucleus 
involving  more  particularly  the  putamen  and  but  slightly  the  globus 
pallidus;  otherwise  speaking,  the  affection  is  an  extra -pyramidal  motor 
disease, 

Clinically  it  is  characterized  by  a  pronounced  spasticity  of  the  limbs 
and  of  the  face  without  or  with  very  little  paralysis;  by  a  bilateral  tremor 
of  all  extremities  which  increases  with  voluntary  movements;  by  dysphagia 
and  dysarthria  or  anarthria;  by  spasmodic  laughing  or  crying  (only  oc- 
casionally) ;  by  absence  of  the  toe  phenomenon  and  preservation  of  the 
abdominal  reflexes;  finally  by  transitory  mental  disturbances;  impair- 
ment of  the  capacity  of  retaining  impressions;  childishness;  docility.     An- 


DISEASES    OF    THE   BASAL    GANGLIA  1 69 

other  characteristic  feature  of  the  disease  is  its  familial  nature.  Finally 
the  disease  is  associated  with  cirrhosis  of  the  liver. 

The  diagnosis  should  be  made  between  this  affection  and  Pseudo- 
bulbar palsy  which  in  some  respects  resembles  the  former.  In  pseudo-bulbar 
palsy  the  palate  and  tongue  are  paralyzed.  In  the  lenticular  disease  there 
is  no  true  palsy  of  these  two  organs.  In  pseudo-bulbar  palsy  there  is  a 
double  hemiplegia  with  the  characteristic  abnormal  reflexes;  they  are  all 
absent  in  the  lenticular  disease.  Pseudo-bulbar  palsy  is  a  disease  of  the 
pyramidal  tract. 

The  tremor  of  the  lenticular  disease  resembles  that  of  Paralysis  agi- 
tans,  but  the  latter  is  a  disease  of  advanced  age  and  chronic  in  nature, 
while  the  lenticular  disease  is  a  disease  of  youth. 

Progressive  lenticular  degeneration  occurs  at  the  age  of  between  10 
and  25,  in  males  more  frequently  than  in  females.  It  is  invariably  fatal. 
Its  duration  is  from  two  to  seven  years.  The  disease  is  probably 
acquired  and  apparently  toxic  in  nature.  That  the  toxin  is  elaborated 
in  the  liver,  is  a  plausible  inference.  Syphilis  and  alcohol  apparently 
play  no  role  whatever. 

Bilateral  softening  of  the  lenticular  nucleus  has  been  observed  in  cases 
of  gas  poisoning.  Dana  (/.  of  Nerv.  and  Ment.  Dis.,  1908)  published 
such  cases.  He  observed  during  life  a  vasomotor  (oedema)  and  gangrenous 
condition  of  the  skin  which  he  considered  belongs  to  the  syndrome  of  the 
corpus  striatum.  He  says  that  this  body  has  no  specific  and  independ- 
ent motor  function.  It  has  a  supplementary  motor  function  and  espe- 
cially in  connection  with  articulation.  It  may  have  some  control  over  the 
bladder.  Gowers,  Ormond,  Homen  and  especially  Mme.  Vogt  published 
cases  in  which  there  is  the  greatest  resemblance  with  the  syndrome  as  given 
by  Wilson.  The  latter's  description  could  be  considered  as  a  classical 
picture  of  the  symptom-group  of  the  corpus  striatum. 

IH.  LESION  OF  THE  CORPORA  QUADRIGEMINA 

The  anterior  quadrigeminal  bodies  are  connected  with  the  optic  tracts, 
and  are  therefore  concerned  in  vision,  the  posterior  bodies  are  connected 
with  the  cortex  of  the  temporal  lobe  and  are  consequently  concerned  in 
hearing  (see  Anatomy.)  The  symptomatology  will  therefore  differ 
according  to  what  tubercles  are  involved.  Diseases  (hemorrhage, 
softening  or  tumor),  confined  exclusively  to  the  quadrigeminal  bodies 
are  exceedingly  rare.  The  adjacent  portions  are  usually  involved  simul- 
taneously. The  symptoms  cannot  therefore  be  uniform.  However  the 
following  symptoms  have  been  observed  almost  constantly  in  tumors 
limited  to  the  quadrigeminal  bodies. 


170  DISEASES    OF   THE   BASAL    GANGLIA 

Eye  Symptoms.- — Ptosis,  paralysis  of  bilateral  associated  movements 
of  the  eyes — upward  and  downward,  also  limitation  of  lateral  movements 
of  the  eyes,  finally  paralysis  of  convergence — these  are  the  most  common 
ocular  phenomena.  Ophthalmoplegia  is  therefore  characteristic.  It  is 
due  to  an  involvement  of  the  oculomotor  nuclei  situated  beneath  the 
quadrigeminal  bodies.  The  pupils  are  usually  unequal  and  dilated. 
Later  in  the  course  of  the  disease  when  secondary  changes  set  in  the 
optic  nerves,  the  vision  becomes  defective,  but  the  latter  is  not  impaired 
in  the  beginning. 

Ear  Symptoms. — When  the  posterior  quadrigeminal  bodies  are  affected 
the  hearing  becomes  deficient.  Some  observations  point  to  the  fact  that 
a  lesion  of  one  posterior  quadrigeminal  body  causes  impairment  of  hearing 
on  the  opposite  side. 

Motor  Symptoms. — Nothnagel  observed  in  almost  all  cases  a  gait 
characteristic  of  an  intoxicated  person  similar  to  that  of  cerebellar 
diseases.  Loss  of  equilibrium  in  standing  is  another  symptom.  Motor 
paralysis  is  absent  except  in  advanced  stages  when  the  tumor  presses  on 
the  motor  tracts.  Nothnagel  claims  that  when  the  cerebellar  gait  appears 
as  the  first  symptom  and  especially  in  association  with  the  above  mentioned 
ocular  phenomena,  the  diagnosis  is  certain.  The  knee-jerks  are  usually 
not  involved  except  when  the  motor  tract  becomes  infringed  upon  by 
the  progress  of  the  tumor.  Then  they  become  exaggerated  and  the 
toe  phenomenon  makes  its  appearance.  They  may  be  found  also  dimin- 
ished or  abolished  like  in  cerebellar  diseases. 

Tremor  of  the  arms,  also  of  the  head  and  legs  has  been  observed  in 
some  cases. 


CHAPTER  XII 

DISEASES  OF  THE  MEMBRANES  OF  THE  BRAIN 
I.  INFLAMMATION  (MENINGITIS) 

In  the  chapter  on  anatomy  we  have  seen  that  the  brain  is  practically 
covered  with  two  membranes,  viz.  dura  and  pia-arachnoid.  An  in- 
flammation of  the  pia  and  that  of  the  arachnoid  separately  does  not  exist; 
both  latter  membranes  are  usually  involved  simultaneously.  We  will 
therefore  consider  an  inflammation  of  the  dura — pachymeningitis,  and  an 
inflammation  of  the  pia-arachnoid— leptomeningitis. 

A.  Pachymeningitis. — The  dura-mater  consists  of  two  layers.  The 
outer  layer,  which  is  intimately  adherent  to  the  skull,  is  considered  as  its 
endosteum.  The  inner  layer  is  smooth  and  in  contact  with  the  arach- 
noid. Either  of  these  layers  may  become  inflamed  independently  of 
each  other.     There  are  therefore  external  and  internal  pachymeningitis. 

(a)  External  Pachymeningitis  is  mainly  a  surgical  affection;  it  is  always 
secondary  to  diseases  of  the  skull  and  especially  to  injuries  of  the  latter. 
When  a  fracture  occurs,  the  blood  detaches  the  outer  layer  of  the  dura. 
The  accumulated  blood  may  become  absorbed  when  it  is  in  a  small  quan- 
tity, but  in  unfavorable  cases  suppuration  takes  place.  Caries  of  the  bone, 
osteitis  may  also  lead  to  an  external  pachymeningitis. 

Pathology. — At  first  red  and  cedematous,  the  dura  soon  becomes  thick- 
ened and  adhesions  form  between  it  and  the  skull.  This  is  frequently  ob- 
served in  lesions  of  the  petrous  bone.  In  some  cases  suppuration  takes 
place  and  purulent  collections  are  found  between  the  dura  and  the  skull. 

Symptoms. — In  severe  traumatic  cases  there  is  usually  coma,  which 
sometimes  may  be  deep  and  prolonged.  In  case  the  blood  or  pus  compress 
any  of  the  cortical  centers,  corresponding  symptoms  will  be  present  (see 
Localizations).     Hemiplegia,  Jacksonian  epilepsy  are  not  infrequent. 

Treatment. — The  only  rational  treatment  is  surgical  intervention, 
which  must  be  instituted  as  promptly  as  possible. 

(b)  Internal  Pachymeningitis. — It  is  frequently  called  hemorrhagic 
internal  pachymeningitis  or  hematoma  of  the  dura.  The  disease  is  very 
rare  and  met  with  in  the  extreme  ages  of  life,  viz.  in  very  young  children 
and  aged  individuals.  It  occurs  not  infrequently  in  the  insane  and  espe- 
cially in  paretics.     Alcoholism  (chronic)  is  a  very  frequent  etiological  factor. 

171 


172  DISEASES    OF    MEMBRANES    OE  BRAIN 

Rheumatic  diathesis,  infectious  diseases,  traumatism  are  other  causes  of 
pachymeningitis  interna.  Purpura,  scurvy,  rickets  and  tuberculosis  are 
the  causes  of  infancy. 

Pathology. — The  chief  lesion  lies  between  the  arachnoid  and  the  dura 
and  consists  essentially  of  a  thickening  of  the  latter  and  formation  of 
layers  of  membranes  on  its  inner  surface  to  which  they  adhere.  The 
membranes  are  very  vascular.  The  new  blood  vessels  of  this  new  tissue 
have  external  and  internal  coats,  but  no  media,  a  condition  which  makes 
them  fragile.  Rupture  of  the  new  blood  vessels,  which  frequently  un- 
dergo degeneration,  is  the  origin  of  the  blood  found  in  pachymeningitis. 
The  blood  accumulates  between  the  laminae  of  the  newly  formed  mem- 
branes. The  amount  of  blood  varies  from  a  very  small  quantity  to  a 
very  large  collection.  The  hemorrhagic  cyst  thus  formed  may  undergo 
transformations,  among  which  the  most  important  are  serous,  purulent 
(through  secondary  infection)  and  state  of  calcification.  It  goes  with- 
out saying  that  the  brain  tissue  will  suffer  from  compression  caused  by 
the  adherent  cyst;  inflammation  with  softening  will  be  the  result.  The 
most  frequent  seat  of  the  hematoma  is  the  parietal  lobe;  then  in  order  of 
frequency  come  the  temporal,  frontal  and  occipital. 

Symptoms. — When  the  hematoma  is  very  small,  the  pachymen- 
ingitis may  pass  unobserved,  but  most  of  the  time  the  hematoma  reaches 
a  sufficient  size  to  present  symptoms  of  cerebral  compression.  In  the 
majority  of  cases  prodromal  symptoms  are  present.  They  are:  headache, 
vertigo  and  a  paralytic  condition,  accompanied  by  convulsions  on  one 
side.  Headache  may  be  the  only  symptom  for  some  time.  It  is  very 
severe,  localized  on  one  side  of  the  skull  and  usually  not  accompanied 
by  fever.  The  pupils  are  as  a  rule  contracted  but  more  on  the  side  of 
the  lesion.  Nystagmus  may  be  present.  (Edema  of  the  papilla  is  fre- 
quently observed  and  it  is  due  to  the  compression;  it  has  the  same  value 
as  in  tumors  of  the  brain  with  this  difference  that  it  appears  late  in  the 
course  of  pachymeningitis.  Gradually  and  sometimes  rapidly  coma 
makes  its  appearance;  at  first  it  is  only  slight,  but  gradually  becomes 
more  and  more  profound.  Death  supervenes  usually  in  a  short  time. 
The  paralysis  may  affect  one  or  two  limbs,  also  the  face.  The  palsy  is 
rarely  complete.     Its  onset  is  slow  but  progressive. 

In  cases  with  a  slow  onset  a  gradually  increasing  mental  hebetude 
with  great  somnolence  are  the  most  striking  features.  In  a  case  of  a 
physician  of  the  Jefferson  Hospital,  who  died  from  this  affection,  there 
was  noticed  an  extraordinary  tendency  to  sleep;  he  would  fall  asleep 
wherever  he  happened  to  be.  I  also  noticed  that  he  was  unable  to  con- 
centrate his  attention.     Gradually  he  developed  unilateral  convulsions, 


DISEASES    OF   MEMBRANES    OF   BRAIN  1 73 

an  exaggerated  knee-jerk  on  the  same  side,  also  the  paradoxical  reflex. 
An  extensive  hematoma  of  the  dura  was  found  on  the  opposite  side. 

Prognosis. — In  all  cases  the  outlook  is  very  grave.  Recovery  is  ex- 
tremely rare.  Remissions  may  occur  in  the  course  of  the  disease,  but 
death  is  the  usual  termination.  The  duration  is  usually  from  a  few  weeks 
to  several  months. 

Diagnosis. — Mental  hebetude,  somnolence,  headache  with  localize 
palsies  and  convulsions  may  be  met  with  in  cerebral  syphilis.  The 
paroxysmal  character  of  the  headache  and  its  aggravation  at  night, 
finally  the  history  of  a  specific  infection  will  differentiate  brain  syphilis 
from  internal  pachymeningitis. 

It  was  mentioned  above  that  the  paralytic  symptoms  develop  gradually 
in  hematoma  of  the  dura.  This  will  differentiate  the  condition  from 
cerebral  hemorrhage,  in  which  the  apoplectic  seizure  and  the  hemiplegia 
appear  suddenly. 

Tubercular  Meningitis  may  sometimes  simulate  internal  pachy- 
meningitis, but  the  less  acute  headache,  constipation,  retraction  of  the 
abdomen  and  the  character  of  the  cerebro-spinal  fluid  of  the  former  will 
render  the  diagnosis  less  difficult.  The  greatest  difficulty  is  experienced 
in  making  a  diagnosis  between  hematoma  of  the  dura  and  cerebral  tumors. 
In  such  cases  it  may  be  of  some  use  to  remember  that  oedema  of  the 
papilla  is  a  very  frequent  occurrence  in  tumors.  It  is  less  frequent  in 
pachymeningitis  and  it  appears  quite  late  in  the  latter  disease. 

Treatment. — Counter-irritation,  leeching  in  the  mastoid  regions, 
elevation  of  the  head,  ice  to  the  head,  avoidance  of  stimulants  are  all  the 
means  used  in  such  cases.  If  there  are  strictly  localized  symptoms, 
evacuation  of  the  hemorrhagic  focus  is  advisable.  In  the  case  mentioned 
above  the  first  operation  gave  an  excellent  result.  The  patient  rallied 
and  the  cephalalgia  disappeared. 

B.  Leptomeningitis. — It  may  be  acute  and  chronic. 

Acute  Meningitis. — In  studying  the  acute  form  the  following  varieties 
will  be  considered:  (i)  Non- tubercular,  (2)  tubercular  and  (3)  epidemic 
cerebro-spinal  meningitis. 

I.  NON-TUBERCULAR  MENINGITIS. 

The  latest  researches  in  the  domain  of  microbiology  (Frankel,  Weich- 
selbaum,  Netter,  etc.)  have  definitsly  established  the  infectious  nature 
of  meningitis.  Meningeal  infection  may  originate  from  an  intra-cranial 
or  extra-cranial  focus.  In  the  first  case  a  cerebral  abscess  will  invade 
the  meninges  by  direct  contact  or  through  the  blood  vessels.     In  the 


174  DISEASES    OF   MEMBRANES    OF  BRAIN 

second  case  a  fracture  of  the  skull  will  present  a  direct  road  for  invasion 
of  the  microbes  from  exterior.  The  natural  cavities  located  at  the  base 
of  the  cranium,  viz.  nasal,  orbital,  pharyngeal,  auricular,  etc.,  which  are 
constantly  exposed  to  infection,  are  in  relation  with  the  cranial  cavity  by 
means  of  a  large  number  of  blood  vessels  and  nerves;  the  infection  can  be 
therefore  easily  transmitted.  Various  diseases  of  these  cavities  may 
easily  lead  to  an  infectious  involvement  of  the  meninges.  Among  them 
the  most  frequent  is  the  infection  of  the  ears.  All  suppurations  of  the 
latter  are  to  be  feared,  especially  those  of  the  middle  ear  which  spread 
to  the  mastoid  cells.  The  infection  is  here  frequently  transmitted  to  the 
meninges  through  the  lymphatic  vessels  or  through  the  neighboring 
sinuses.  An  infection  may  originate  on  the  face  (abscess,  erysipelas, 
a  boil,  etc.),  in  the  hair,  in  the  bones  of  the  cranium  (osteomyelitis, 
tuberculosis  or  syphilis) .  An  infection  of  any  part  of  the  body  may  become 
the  point  of  departure  of  meningitis.  The  latter  may  develop  in  the  course 
of  infectious  diseases  (pneumonia,  typhoid  fever,  etc.)  and  present  there- 
fore a  bronchial,  pulmonary,  intestinal,  biliary,  urinary,  endocardial  or 
puerperal  origin.  In  such  cases  the  microbes  or  their  toxins  pass  into 
the  general  circulation  and  the  meninges,  which  are  exceptionally  rich  in 
blood  vessels,  become  therefore  easily  the  seat  of  secondary  infection. 

The  largest  number  of  cases  of  meningitis  are  due  to  the  pneumococcus 
(Netter).  It  may  develop  secondarily  to  a  pneumococcic  infection  or 
primarily.  In  the  latter  case  it  may  be  sporadic  or  epidemic.  The 
gross  characteristic  feature  of  meningitis  due  to  this  microbe  is  the  forma- 
tion of  pus,  which  is  greenish  and  particularly  thick. 

Other  microbes  are  met  with  less  frequently.  In  order  of  frequency 
they  are:  Meningococcus  of  Weichselbaum,  diplococcus  of  Talamon- 
Frankel,  streptococcus,  typhoid  bacillus,  staphylococcus.  There  are 
also  cases  in  which  the  meningitis  is  due  to  a  mixed  infection,  as  for 
example,  pneumococcus  with  staphylococcus  and  others.  It  is  impos- 
sible at  present  time  to  differentiate  clinically  with  precision  the  forms 
of  meningitis  caused  by  various  microbes.  They  all  have  many  common 
features.  The  predisposing  factors  should  not  be  overlooked  in  cases 
of  meningitis.  Traumatism,  exposure  to  cold,  intellectual  exhaustion 
and  alcoholism,  are  all  conditions  which  favor  an  invasion  of  microor- 
ganisms. 

Finally  meningitis  may  occur  independently  of  microorganisms. 
Such  is  the  case  of  lead  intoxication.  Meningitis  in  chronic  lead  poison- 
ing is  an  established  fact. 

Pathology. — The  first  period  of  an  inflammatory  condition  of  the  pia- 
arachnoid  is  characterized  by  a  congestion.     The  meninges  are  red,  the 


DISEASES    OF   MEMBRANES    OF  BRAIN  1 75 

blood  vessels  are  dilated  not  only  in  the  membranes,  but  also  in  the 
adjacent  portion  of  the  cortex.  Soon  exudation  takes  place.  The 
latter  may  be  serous,  or  purulent.  The  fluid  of  the  serous  exudate 
differs  from  cerebro-spinal  fluid  in  that  it  contains  a  larger  quantity  of 
albumen  and  of  leucocytes.  Quite  frequently  fibrinous  masses  form  in 
the  exudate;  they  He  over  and  between  the  convolutions,  agglutinate  the 
latter  to  each  other  and  raise  the  arachnoid.  Such  are  the  findings  in 
meningitis  in  its  early  stages.  In  an  advanced  stage  pus  takes  the  place 
of  the  serofibrinous  exudate.  Most  frequently  it  is  arranged  in  bands  or 
else  in  islands.  Sometimes  it  is  so  abundant  that  it  covers  the  entire 
convexity  of  both  hemispheres,  extends  to  the  base,  envelops  the  medulla 
and  involves  the  cranial  nerves.  Between  the  serous  and  purulent 
exsudates  there  are  intermediary  varieties,  as  the  transition  of  one 
into  the  other  is  not  rapid. 

The  pia-mater  is  infiltrated  and  cedematous.  The  inflammation 
usually  spreads  to  the  choroid  plexus  and  to  the  ventricles.  The  choroid 
plexuses  are  swollen,  thick  and  frequently  covered  with  pus.  The  con- 
tents of  the  ventricles  may  be  transparent,  purulent  or  sero-purulent  or 
else  hemorrhagic.  The  brain  tissue  is  involved  to  a  more  or  less  great  degree 
according  to  the  stage  of  meningitis.  At  an  early  period,  when  there  is 
only  a  serous  exudate,  small  hemorrhagic  foci  with  abundant  leucocytes 
will  be  noticed.  In  an  advanced  stage  softening  of  cerebral  tissue  is 
observed.     It  is  due  to  vascular  changes  in  the  affected  area. 

Symptoms. — The  many  etiological  factors  enumerated  above  will 
naturally  lead  to  the  idea  that  there  may  be  a  great  variety  of  clinical 
pictures  of  acute  meningitis.  Nevertheless,  irrespective  of  the  nature  of 
meningeal  infection  there  exists  a  general  type  in  which  the  symptoms  are 
met  with  constantly  and  from  which  the  special  forms  differ  little.  The 
symptoms  of  this  type  are  as  follows. 

Two  distinct  phases  are  observed  in  the  majority  of  cases,  viz. 
excitation  and  depression.  Very  frequently  the  first  period  begins 
almost  suddenly  by  an  intense  fever  and  a  chill.  In  other  cases  the 
onset  is  insidious  and  preceded  for  a  few  days  by  headache  and  vomiting. 
In  children  the  onset  may  be  accompanied  by  convulsions. 

Gradually  the  initial  symptoms  become  aggravated.  The  headache 
which  is  continuous  assumes  an  unusually  severe  character :  it  is  burning, 
lancinating,  increased  upon  the  slightest  motion,  a  noise  in  the  vicinity 
of  the  patient  or  upon  exposure  to  light.  Insomnia  is  the  usual  conse- 
quence of  the  headache.  The  fever  which  is  from  the  first  pronounced 
(ioi°-io3°)  remains  as  such  throughout  the  disease,  although  morning 
remissions  may  occur.     In  the  purulent  form  of  meningitis  the  tempera- 


176  DISEASES    OF   MEMBRANES    OF  BRAIN 

ture  may  reach  1040  or  1050.  In  cases  of  fatal  termination  the  tempera- 
ture may  ascend  even  to  1060  or  1080  toward  the  end.  The  fever  is 
attended  by  a  rapid  and  full  pulse.  Absence  of  parallelism  of  pulse 
and  temperature,  viz.  elevation  of  temperature  and  slow  pulse  or  vice  versa,  is 
observed  quite  frequently  in  all  forms  of  meningitis  but  especially  in  the 
tubercular  form.  The  respiration  is  accelerated  and  irregular :  the  thoracic 
and  diaphragmatic  movements  are  not  synchronous  during  breathing. 
Vomiting  is  of  a  cerebral  character,  viz.  it  occurs  without  the  slightest 
effort  and  unaccompanied  by  nausea.  Constipation  is  unavoidably  pres- 
ent. It  is  unusually  rebellious  to  purgatives.  The  abdomen  is  retracted. 
The  facies  of  the  patient  is  quite  characteristic:  redness  and  pallor  alter- 
nate, the  eyes  are  glossy,  photophobia  is  marked.  The  skin  is  warm  and 
dry.  Irritation  of  the  skin  with  a  nail  is  followed  by  a  persistent  redness 
(Taches  cerebrales). 

Ocular  phenomena  are  particularly  important.  At  first  strabismus 
with  diplopia  makes  its  appearance.  The  pupils  are  unequal  and  con- 
tracted but  later  become  dilated.  The  eye-grounds  show  a  dilatation  of 
the  retinal  veins  and  lateral  oedema  of  the  papillae. 

Optic  neuritis  is  a  frequent,  although  not  a  constant  symptom,  espe- 
cially in  meningitis  of  the  base  of  the  brain.  Other  cranial  nerves,  par- 
ticularly those  of  the  ocular  muscles  and  the  facial  are  frequently  affected 
in  basal  meningitis. 

The  symptoms  of  excitation  which  characterize  the  first  period  affect 
the  intellectual  sphere,  the  motor  and  sensory  apparatus. 

Delirium  appears  with  the  early  symptoms.  The  patient  is  agitated, 
very  talkative,  restless.  He  talks  incoherently,  rapidly  and  loudly, 
screams,  shouts.  He  fights,  resists  restraint.  Hallucinations,  especially 
visual,  are  frequent. 

The  motor  symptoms  are  manifested  in  convulsions,  palsies  and  con- 
tractures. The  first  are  particularly  frequent  in  children.  They  may  be 
generalized  or  localized.  Contractures  are  more  frequent  than  convul- 
sions. They  are  almost  constant  and  have  an  important  diagnostic  value. 
They  are  very  irregular  and  intermittent.  The  most  frequent  seat  of 
contractures  is  in  the  muscles  of  the  neck  (posterior  cervical  muscles). 
The  rigidity  of  the  neck  is  the  earliest  and  the  most  persistent  phenomenon; 
it  affects  particularly  the  movements  of  flexion  of  the  head.  If  the  muscles 
of  the  back  are  involved,  opisthotonos  will  be  the  result.  The  muscles  of 
mastication  (trismus),  of  the  eye  globes  (strabismus),  of  the  face,  of  the 
pharynx,  of  the  larynx,  of  the  tongue,  of  the  abdomen  and  of  the  sphincters 
may  be  affected.  In  the  limbs  the  contracture  affects  mostly  the  flexor 
muscles.     The  presence  of  contracture  can  be  shown  by  Kernig's  sign.    It 


DISEASES    OF   MEMBRANES    OF  BRAIN  1 77 

consists  of  an  inability  to  extend  the  leg  over  the  thigh  when  the  patient 
is  sitting,  or  else  when  the  patient  is  lying  on  his  back  with  his  thighs 
flexed  over  the  trunk.  This  phenomenon  is  due  to  an  irritation  or  inflam- 
mation of  the  spinal  meninges  and  the  roots.  While  it  is  present  almost 
in  every  case  of  meningitis,  nevertheless  it  was  found  in  conditions  other 
than  meningitis,  as  for  example,  in  typhoid  fever;  it  is  absent  in  tubercular 
meningitis.  Brudzinski  {Arch,  de  mid.  des  enfants,  1910)  described  a 
neck  reflex  which  is  constant  in  meningitis.  It  consists  of  flexion  of  the 
thighs  and  legs,  when  the  head  is  firmly  flexed  forward. 

Among  other  motor  symptoms  can  be  mentioned  exaggerated  deep  and 
superficial  reflexes.  The  plantar  reflex  appears  in  extension  on  one  side 
and  in  flexion  on  the  other.  The  knee-jerks  are  sometimes  abolished. 
Paralysis  of  the  extremities  may  appear  quite  early  in  meningitis.  It 
may  be  only  transitory  and  disappears  when  the  patient  commences  to 
improve,  or  it  may  develop  gradually  and  remain  permanent.  The  par- 
alysis may  be  hemiplegic  or  monoplegic;  the  face  is  usually  also  involved. 

The  sensory  symptoms  of  the  first  phase  of  meningitis  consist  of 
general  cutaneous  hyperesthesia  and  disturbances  of  the  special  senses 
(hearing  and  sight) ;  the  least  noise  is  painful  to  the  patient;  photophobia  is 
a  common  observation. 

When  the  patient  enters  the  second  phase  of  the  disease  the  above 
described  symptoms  gradually  become  less  and  less  pronounced.  De- 
pression takes  the  place  of  the  tumultuous  manifestations  of  the  first 
period.  The  delirium  and  agitation  disappear.  The  convulsions  and 
the  contractures  leave  the  patient  and  instead  of  them  paralysis  develops. 
It  may  be  hemiplegic  or  monoplegic.  The  sphincters  of  the  bladder  and 
rectum  also  become  paralyzed;  incontinence  is  the  consequence.  The 
fever  increases,  the  pulse  is  slow.  Mental  hebetude  becomes  more  and 
more  marked,  general  anaesthesia  makes  its  appearance.  Soon  bulbar 
symptoms  enter  the  scene.  The  respiration  becomes  superficial  and  of 
Cheyne-Stokes'  type.  The  extremities  and  face  are  cold  and  death  follows 
in  deep  coma. 

Course.  Duration.  Termination. — Ordinarily  the  general  type  of 
acute  non-tubercular  meningitis  has  a  rapid  course.  In  the  majority  of 
cases  it  lasts  about  eight  or  ten  days.  In  some  cases  death  may  occur  in 
the  first  phase,  during  the  convulsive  period.  While  death  is  the  usual 
termination,  nevertheless  there  are  cases  of  recovery.  The  latter  may  be 
incomplete  or  complete.  In  the  first  case  some  permanent  lesion  may 
be  present  as  a  sequel,  as  for  example,  paralysis,  mental  impairment, 
hydrocephalus,  ocular  palsies. 

Clinical  Forms. — There   are   certain  forms   of  acute   non-tubercular 


178  DISEASES    OF   MEMBRANES    OF  BRAIN 

meningitis  which  differ  more  or  less  from  the  classical  type  described 
above. 

Serous  Meningitis  is  characterized  by  considerably  less  marked 
symptoms.  Headache  and  constipation  are  usually  absent  or  very 
mild.  Delirium  is  less  persistent  than  in  the  purulent  form.  According 
to  Quincke  congestion  of  the  retina  is  a  frequent  occurrence.  The  course 
is  long  and  irregular  with  periods  of  amelioration  and  aggravation. 
Recovery  is  the  usual  outcome.  The  nature  of  serous  meningitis  deserves 
special  mention.  Bacteriological  researches  of  the  cerebro-spinal  fluid 
have  demonstrated  either  presence  or  absence  of  microorganisms.  Ty- 
phoid bacillus,  pneumococcus,  staphylococcus,  Pfeiffer's  bacillus,  have 
been  found.  Serous  meningitis  is  therefore  of  an  infectious  origin.  The 
same  results  have  been  obtained  experimentally  by  injecting  various 
cultures  into  the  meninges.  It  is  consequently  legitimate  to  admit 
an  etiological  unity  of  all  forms  of  acute  meningitis  and  that  bacterial 
infection  of  meninges  may  produce  either  a  plain  congestion  with  serum 
exsudate  or  a  purulent  meningitis.  The  result  is  identical  with  what  is 
observed  in  infections  of  serous  membranes  of  other  organs.  The  fact 
that  sometimes  the  microorganisms  are  not  detected  in  the  cerebro-spinal 
fluid  does  not  speak  against  the  infectious  nature  of  serous  meningitis, 
because  in  some  cases  their  number  is  exceedingly  small  or  they  may  have 
already  disappeared  from  the  fluid  which  is  to  them  a  poor  medium. 
Under  the  name  of  Meningism  Dupre  in  1894  described  slight  meningeal 
manifestations  while  at  autopsy  no  anatomical  changes  are  found.  They 
may  develop  either  primarily  (in  hysteria,  or  in  association  with  intes- 
tinal parasites)  or  secondarily  in  the  course  of  infectious  diseases.  Prob- 
ably all  those  cases  of  so-called  meningismus  are  in  reality  curable  cases 
of  slight  serous  meningitis. 

Circumscribed  Serous  Meningitis  was  first  described  by  Strobe  in 
1904.  It  is  met  with  in  cases  in  which  a  former  meningitis  produced 
adhesions  and  thus  isolated  an  arachnoid  area.  It  consists  of  an  infiltra- 
tion with  fluid  of  the  arachnoid  tissue  which  is  therefore  distended.  It 
is  a  sort  of  cedema  of  this  tissue  and  the  fluid  is  imprisoned  in  it.  The 
fluid  is  clear  and  sterile  and  is  under  sufficient  tension  to  compress  the 
underlying  nervous  tissue  and  thus  produce  symptoms  of  a  neoplasm. 
The  most  frequent  seat  of  these  pseudo-tumors  is  the  spinal  region. 
The  latter  was  particularly  studied  by  Schlesinger  and  Krause.  In  the 
cerebellar  region  they  are  more  frequent  than  in  the  cerebrum.  The 
nature  of  circumscribed  serous  meningitis  is  probably  infectious  in  spite 
of  the  fact  that  the  cerebro-spinal  fluid  is  found  to  be  aseptic.  The  diag- 
nosis between  this  affection  and  tumor  is  difficult.     Oppenheim  mentions 


DISEASES    OF    MEMBRANES    OF   BRAIN  1 79 

alternating  remissions  and  aggravation  as  a  characteristic  diagnostic 
sign.  The  treatment  consists  of  excision  of  the  wall  of  the  entire  pseudo- 
cyst and  recovery  is  frequently  complete. 

Meningitis  due  to  Pneumococcus. — It  is  the  most  important  form  of 
meningitis.  It  is  encountered  not  only  in  pneumonia,  but  also  in  various 
affections,  such  as  grippe,  typhoid  fever,  endocarditis,  puerperal  fever. 
This  frequency  is  due  to  the  constant  presence  of  the  pneumococcus  in 
the  cranial  cavities.  For  this  reason  traumatic  meningitis  is  frequently 
caused  by  the  pneumococcus.  The  same  germ  is  found  in  meningitis 
complicating  otitis.  In  the  majority  of  cases  this  form  of  meningitis 
is  purulent  and  the  pus  is  situated  between  the  pi  a  and  the  convolutions. 
In  the  primary  form  the  temperature  rises  very  high,  higher  than  in  the 
epidemic  cerebro-spinal  form.  Albuminuria  is  marked.  In  the  secondary 
form  it  usually  remains  latent  and  then  suddenly  a  rise  of  temperature 
with  a  violent  delirium  announces  a  meningeal  complication.  The  cere- 
bro-spinal fluid  shows  usually  leucocytosis  of  the  polynuclear  variety; 
in  some  cases  a  lymphocytosis  precedes  polynucleosis.  Sometimes  no 
leucocytes  can  be  found. 

Typhoid  Meningitis. — Developed  in  the  course  of  typhoid  fever  it  pre- 
sents these  peculiarities:  absence  of  headache,  vomiting  and  constipation. 

The  elevation  of  temperature  is  not  high  and  the  course  is  slow.  It  is 
more  frequent  in  children.  It  usually  develops  in  the  second  week  of 
typhoid  fever.  The  cerebro-spinal  fluid  possesses  a  very  marked  agglutina- 
tive power  for  the  Eberth's  bacillus  contrary  to  ordinary  typhoid  fever  in 
which  this  power  is  none.  Leucocytes  and  microbes  may  be  present  even 
when  the  cerebro-spinal  fluid  is  perfectly  clear. 

Meningitis  in  the  course  of  typhoid  fever  may  not  be  caused  by  typhoid 
bacillus,  but  by  other  microorganisms.  On  the  other  hand  meningitis 
caused  by  the  typhoid  bacillus  may  develop  without  typhoid  fever.  It 
is  then  a  purulent  meningitis. 

The  prognosis  in  typhoid  meningitis  is  various :  it  may  be  favorable  or 
unfavorable.  Netter  claims  that  it  is  unfavorable  when  Kernig's  sign  is 
present. 

Grippal  Meningitis. — In  the  course  of  grippe  meningeal  symptoms  may 
develop.  They  are  not  due  exclusively  to  the  Pfeiffer's  microorganism, 
but  to  association  of  several  germs.  Meningitis  with  Pfeiffer's  bacillus 
alone  is  met  with  in  very  young  children. 

Traumatic  Meningitis. — It  is  caused  by  penetration  of  microorganisms 
either  through  the  cranial  cavities  or  through  the  fractured  skull.  The 
onset  is  characterized  by  elevation  of  temperature  and  rapidity  of  pulse. 
Lumbar  puncture  shows  a  bloody  cerebro-spinal  fluid.     If  centrifugation 


l8o  DISEASES    OF    MEMBRANES    OF   BRAIN 

is  done  immediately,  the  fluid  above  the  blood  will  be  colored  red  if  menin- 
gitis is  present,  but  yellow  if  meningitis  is  absent.  The  sediment  will 
show  a  polynucleosis  90  per  cent,  and  microorganisms. 

The  prognosis  is  not  absolutely  fatal. 

Otitic  Meningitis. — Inflammation  of  the  ears  may  give  rise  to  a 
generalized  or  to  a  localized  meningitis.  Frequently,  but  not  always,  acute 
otitis  causes  a  generalized  meningitis,  while  a  localized  abscess  is  the  result 
of  a  chronic  otitis.  In  children,  meningitis  may  occur  while  the  otitis  is 
apparently  mild.  It  is  an  inflammation  of  the  inner  ear  that  gives  the 
largest  percentage  of  meningitis.  Lermoyez  finds  one  case  of  meningitis 
among  every  eight  cases  of  labyrinthian  disease,  while  in  cases  of  middle 
ear  diseases  the  percentage  is  1  to  600.  Involvement  of  the  labyrinth  can 
be  recognized  by  the  following  symptoms:  noises,  loss  of  hearing,  vertigo, 
nausea;  loss  of  mastoid  perception  of  the  tuning  fork;  vestibular  nystagmus 
especially  on  temperature  tests.  Involvement  of  the  meninges  can  be 
recognized  by  sudden  elevation  of  temperature  which  is  in  contrast  with  a 
slow  pulse,  by  facial  or  oculomotor  palsy,  by  excruciating  pain  in  the 
temporal  region. 

Various  microorganisms  have  been  found.  The  most  frequent  are: 
streptococcus  and  staphylococcus.  As  to  the  cerebro-spinal  fluid,  it  may 
be  clear,  cloudy  or  purulent.  The  appearance  of  the  fluid  is  not  in 
proportion  with  the  gravity  of  the  meningitis,  as  perfectly  clear  fluid  may 
contain  virulent  microbes.  It  has  also  been  shown  that  in  rapidly  fatal 
otitic  meningitis  there  may  be  only  a  congestion  or  oedema  of  the  meninges 
and  brain. 

Syphilitic  Meningitis. — Acute  syphilitic  meningitis  is  rare.  It  usually 
develops  gradually.  We  find  here  the  usual  picture  of  acute  meningitis. 
Palsies  are  very  frequent  and  affect  particularly  the  ocular  muscles  and 
especially  those  supplied  by  the  third  nerve.  The  palsies  are  usually 
transient.  Another  characteristic  feature  is  the  persistent  headache  and 
absence  of  fever. 

Finally  the  evolution  of  the  disease  is  irregular  and  variable.  It  is 
amenable  to  treatment  and  improvement  follows  rapidly,  but  it  has  a  tend- 
ency to  recur.  A  very  important  aid  in  diagnosis  of  syphilitic  meningitis 
is  found  from  a  lumbar  puncture.  The  cerebro-spinal  fluid  shows  a  marked 
lymphocytosis  and  what  is  especially  valuable  is  a  positive  Wassermann 
reaction.  Anatomically  is  seen  a  gelatinous  exsudate  in  the  meninges  in 
which  the  spirochetal  are  found.  The  blood  vessels  show  an  endo-  and 
peri-arteritis. 

Gonococcal  Meningitis. — That  this  microorganism  is  capable  to  pro- 
duce meningitis  is  now  well  established.     The  difficulty  of  recognizing  is 


DISEASES    OF    MEMBRANES    OF   BRAIN  l8l 

the  resemblance  of  the  gonococcus  to  the  meningococcus.  In  a  case  seen 
recently  by  me,  the  resemblance  was  striking.  The  absence  of  agglutina- 
tion of  the  germ  by  antimeningococcus  serum  (precipitin  reaction  of  Vin- 
cent) and  failure  of  the  latter  to  improve  the  patient  decided  the  diagnosis. 
The  patient  had  a  severe  attack  of  gonorrhea  in  the  course  of  which  he 
developed  the  meningeal  symptoms.  A  lumbar  puncture  revealed  a 
slightly  cloudy  fluid  with  abundant  polymorphonuclear  cells  and  the  diplo- 
cocci  in  pairs  with  their  opposed  surfaces  flattened  were  found.  In  spite 
of  repeated  injections  of  serum  the  patient  died. 

Meningitis  of  old  age  presents  some  special  features.  Considering 
the  onset  and  the  course  Schlesinger  {Neurol.  Centralbl.,  No.  20,  191 2)  finds 
the  following  main  forms  of  meningitis  in  aged: 

(1)  Meningitis  with  the  classical  symptoms. 

(2)  Latent  or  ambulatory  type  with  vague  disturbances  assuming  the 
picture  of  neuralgia. 

(3)  Cases  with  apoplectiform  onset  followed  or  not  by  hemiplegia. 

(4)  Meningitis  under  the  picture  of  rapidly  oncoming  dementia. 

As  to  the  symptomatology — rigidity  of  the  lumbar  and  dorsal  spine 
appears  early,  while  rigidity  of  the  neck  is  rarely  an  early  symptom; 
psychic  disturbances  are  almost  invariably  present;  Kernig's  sign  is 
present. 

The  disease  has  usually  a  chronic  course.     It  lasts  weeks. 

The  prognosis  is  favorable.  Sometimes  the  recovery  is  only  clinical 
but  not  anatomical;  irritation  of  the  meninges  may  persist.  The  cerebro- 
spinal fluid  in  long  standing  cases  is  rich  in  albumen  but  poor  in  cells. 

Meningitis  in  alcoholics  is  sometimes  in  a  latent  state,  so  that  sudden 
death  may  occur  very  unexpectedly.  Usually  all  the  symptoms,  and  espe- 
cially the  delirium,  are  at  their  maximum. 

Prognosis  of  Meningitis. — Generally  speaking  it  is  serious.  With 
our  present  methods  of  treatment,  especially  sero-therapy,  recoveries  are 
not  rare.  Frequently  traces  of  the  old  affection,  such  as  palsies,  deafness, 
blindness,  etc.)  remain  indefinitely.  Sometimes  an  apparent  recovery 
may  be  only  a  remission. 

Diagnosis. — When  a  group  of  symptoms  as  described  above  is  observed 
in  a  diseased  individual,  the  first  thought  should  be  that  of  meningitis. 
Nevertheless  we  must  not  lose  sight  of  the  fact  that  purely  cortical  affec- 
tions of  the  brain  and  of  the  cranial  nerves  may  present  an  identical  clinical 
picture.  Also  there  are  cases  on  record  which  presented  the  same  mani- 
festations and  at  autopsies  no  trace  of  meningeal  inflammation  was  dis- 
coverable. In  another  series  of  cases  only  oedema  or  slight  hyperemia  of 
the  meninges  were  found  post-mortem.     The  term  pseudomeningitis  or 


1 82  DISEASES    OF   MEMBRANES    OF  BRAIN 

meningisme  was  created  by  Dupre  to  designate  such  a  condition  (see 
Serous  Meningitis).  Investigations  have  shown  that  in  this  so-called 
meningitis  without  a  lesion,  the  fluid  of  the  oedema  contained  pathogenic 
microorganisms  of  the  disease  during  which  the  meningeal  symptoms 
developed.  The  latter  are  therefore  due  to  the  microbes  or  to  their  toxins. 
Thus  the  examination  of  the  cerebro-spinal  fluid  becomes  an  absolute 
necessity. 

Lumbar  puncture  inaugurated  by  Quincke  in  1891  consists  of  pene- 
trating into  the  spinal  canal  between  thefourth  and  fifth  lumbar  vertebras 
for  the  purpose  of  obtaining  a  small  quantity  of  the  cerebro-spinal  fluid. 
The  examination  of  the  latter  must  be  physical,  chemical  and  bacterio- 
logical. The  following  are  the  characteristic  features  of  this  fluid  in  acute 
meningitis. 

The  color  is  cloudy  or  purulent  and  sometimes  bloody.  The  presence 
of  pus,  microbes  or  tubercle  bacillus  is  an  indisputable  indication  of  menin- 
gitis. A  clear  fluid,  without  changes  in  its  contents,  is  most  frequently 
(but  not  always)  an  indication  of  absence  of  meningeal  involvement.  In 
one  of  my  cases  of  acute  meningitis  the  cerebro-spinal  fluid  obtained  from 
a  lumbar  puncture  was  perfectly  normal  while  at  autopsy  the  convexity  of 
the  brain  was  covered  with  pus. 

The  following  findings  permit  to  establish  a  diagnosis  with  great 
probability. 

When  the  fluid  is  purulent,  non-tubercular  meningitis  is  almost  certain. 

When  the  fluid  is  bloody  and  the  blood  is  of  a  pathological  origin  (not 
due  to  the  puncture  of  the  skin)  it  will  remain  bloody  during  the  lumbar 
puncture  from  the  beginning  to  end.  After  standing,  the  blood  will  form  a 
sediment,  and  the  upper  layer  will  be  a  transparent  fluid  of  yellow  color. 
The  latter  is  characteristic  of  intraspinal  hemorrhage. 

When  the  fluid  is  clear,  meningeal  involvement  will  be  revealed  by  the 
following  symptoms :  if  the  fluid  leaves  the  spinal  canal  under  great  pres- 
sure; if  after  standing  several  hours  or  less  a  thread-like  formation  is  seen 
and  finally  if  there  is  an  increase  of  albumen  (normally  there  are  only  traces 
of  it).  If  the  latter  two  are  present,  tubercular  meningitis  is  almost  in- 
variably certain.  Cryoscopical  study  shows  that  the  freezing  point  is 
lowered  and  oscillates  between  0.490  and  0.640  (normally  it  is  o.72°-o.75°). 
The  most  important  information  is  obtained  from  a  cytological  and  bac- 
teriological examination.  The  polynuclear  leucocytes  predominate  in 
acute  non-tubercular  meningitis,  while  in  the  tubercular  form  the  lympho- 
cytes are  in  abundance  (normally  only  a  few  cells  are  found).  This  leu- 
cocytic  formula  is  not  absolute  in  every  case;  the  condition  may  be  found 
reversed.     Besides,  lymphocytosis  may  be  observed  in  other  affections, 


DISEASES    OP   MEMBRANES    OE  BRAIN  1 83 

such  as  tumors  or  abscess  of  the  brain.  Polynucleosis  may  be  found  in 
tubercular  meningitis.  In  all  cases  cytological  examination  is  not  the 
only  one  to  be  relied  upon.  On  the  other  hand  the  absence  of  the  leuco- 
cytic  formula  is  not  a  sufficient  indication  to  reject  the  diagnosis  of  menin- 
gitis. Danielopolu  and  Iancovescu  (C.  R.  Societe  de  Biologie,  1911)  have 
described  a  reaction  which  is  based  on  the  following  principle:  "the  cere- 
brospinal fluid  in  normal  condition  interferes  to  a  certain  extent  with  the 
hemolytic  action  of  taurocholate  of  sodium."  In  cases  of  meningitis  this 
special  characteristic  is  very  much  pronounced.  The  reaction  was  posi- 
tive in  all  cases  of  meningitis,  but  negative  in  other  diseases  of  the  ner- 
vous system.  It  has  therefore  a  diagnostic  importance  especially  in  cases 
with  a  negative  or  doubtful  leucocytic  formula. 

A  bacteriologic  examination  is  necessary  in  all  cases.  It  reveals  espe- 
cially during  the  first  period  of  the  malady  various  microorganisms: 
pneumococcus,  diplococcus,  streptococcus,  etc.  As  the  cerebro-spinal 
fluid  is  a  poor  culture-medium,  it  is  necessary  to  make  cultures  and  in- 
oculations. The  presence  of  microorganisms  decides  the  nature  of  the 
meningitis.  One  must  bear  in  mind  that  the  search  for  tubercular  bacilli 
is  not  always  successful. 

Wassermann  test  is  another  valuable  addition  to  the  above  mentioned 
investigations  of  the  cerebro-spinal  fluid.  When  it  is  found  positive,  the 
syphilitic  nature  of  the  meningitis  cannot  be  doubted.  As  to  the  spiro- 
chsetas  I  have  never  succeeded  in  locating  them  in  this  fluid. 

Recently  Vincent  and  Bellot  (Presse  Med.,  191 1)  have  devised  a  new  test 
for  diagnosing  meningitis  caused  by  meningococcus  and  meningitis  by 
other  microorganisms.  The  test  which  is  called  '  precipitin  reaction" 
consists  of  adding  one  or  two  drops  of  antimeningococcus  serum  to  a  tube  of 
freshly  obtained  cerebro-spinal  fluid  which  has  been  cleared  by  centrifuga- 
tion  for  ten  minutes;  the  test-tube  is  then  placed  in  an  incubator  at  520  C. 
(125. 6°  F.)  for  a  few  hours.  In  case  of  meningococcus  meningitis  precipi- 
tation will  be  present.  This  reaction  is  very  important  as  it  determines 
the  therapeutic  conduct:  when  it  is  positive,  antimeningococcus  serum 
should  be  used. 

The  diagnosis  of  acute  meningitis  presents  sometimes  difficulties  in 
differentiating  from  cerebral  abscess  following  otitis  media.  The  localized 
symptoms  and  the  character  of  the  headache  will  in  the  majority  of  cases 
decide  the  question  (see  Cerebral  Abscess). 

(•  Tumors  of  the  brain  show  usually  localizing  symptoms,  of  which  focal 
epilepsy  is  the  most  frequent.  Besides,  there  is  no  elevation  of  temper- 
ature and  the  course  is  very  much  more  prolonged  than  in  meningitis. 

Tubercular  Meningitis  will  be  recognized  by  its  subacute  course, 


184  DISEASES    OE   MEMBRANES    OE  BRAIN 

less  intense  fever  and  delirium  and  longer  duration;  finally  by  the  existence 
of  some  tubercular  focus  and  by  the  cytological  examination  of  the  cerebro- 
spinal fluid. 

Cerebro-spinal  meningitis  will  be  recognized  by  the  presence  of  the 
meningococcus  in  the  cerebro-spinal  fluid  and  by  Vincent's  precipitin 
reaction. 

Treatment. — The  frequency  of  fatal  termination  of  the  disease  shows 
the  failure  of  therapeutic  measures.  The  treatment  is  therefore  only 
symptomatic:  antipyretics,  purgatives,  application  of  ice  to  the  head, 
sedatives,  narcotics  are  the  only  means  at  our  command.  Mercurial 
treatment  should  be  tried  in  syphilitic  cases.  Bleeding  and  counter-irri- 
tants should  be  avoided,  as  they  weaken  the  organism.  Bathing  is  useful. 
Warm  baths  have  a  more  beneficial  effect  than  cold:  they  decrease  the 
nervousness,  relieve  pain  and  contractures,  produce  diuresis.  Saline  in- 
fusions may  be  of  benefit.  In  a  case  of  a  child  under  my  care  remarkable 
immediate  results  were  obtained  from  the  latter  procedure  (Therap. 
Gazette,  1902). 

Lumbar  puncture  with  evacuation  of  a  certain  amount  of  cerebro- 
spinal fluid  relieves  considerably  intracranial  hypertension  and  therefore 
relieves  the  headache.  In  some  cases  a  prolonged  evacuation  has  given 
satisfactory  results :  the  needle  is  left  in  the  spinal  canal  for  several  hours, 
but  this  procedure  is  not  without  some  danger.  As  to  injections  of  anti- 
meningococcus  serum  this  will  be  discussed  later. 

Haines  {Transactions  of  the  Ninth  Internat.  Otological  Congress,  191 2) 
has  recently  devised  a  new  surgical  procedure  in  treatment  of  meningitis  of 
any  type  of  the  disease  except  the  meningococcus  variety.  If  a  free  and 
continuous  drainage  could  be  established  for  the  cerebro-spinal  fluid,  the 
intracranial  pressure  in  meningitis  would  subside  and  the  exsudate  be  reab- 
sorbed. As  in  meningitis  the  excess  of  cerebro-spinal  fluid  crowds  the  brain 
against  the  skull,  no  operation  could  be  successful  when  performed  on  the 
convexity  of  the  brain:  as  soon  as  the  brain  tissue  is  exposed,  it  would  crowd 
into  the  opening  and  become  lacerated.  In  such  cases  no  drain  introduced 
could  functionate  properly.  Haines  argues  that  the  space  between  the  two 
poles  of  the  cerebellum  and  the  medulla,  the  cisterna  magna,  is  in  free  com- 
munication with  all  other  subarachnoid  spaces  about  the  brain  and  cord 
and  with  the  cerebral  ventricles.  This  space  is  most  accessible  to  surgical 
attack.  Opening  of  this  space  is  not  followed  by  a  hernia  cerebelli.  A 
drainage  established  here  is  free  and  continuous.  The  operation  consists 
of  an  incision  in  the  mid-line  from  the  occipital  protuberance  to  the  spinous 
process  of  the  axis.  A  trephine  is  applied  about  an  inch  above  the  margin 
of  the  foramen  magnum  in  the  mid-line.     If  the  occipital  sinus  is  found 


DISEASES    OF    MEMBRANES    OF  BRAIN  185 

single,  it  is  tied  up  at  its  upper  part.  If  double,  incision  of  the  dura  is 
made  between  them.  After  the  dura  is  incised  and  a  certain  amount  of 
fluid  allowed  to  escape,  a  drain  (gutta-percha)  is  introduced.  Haines 
contends  that  if  this  operation  is  performed  early,  a  cure  may  be  obtained. 

This  operation  may  prove  of  value  also  in  basal  fractures. 

Preventive  Measures  should  never  be  neglected  in  cases  of  lesions  of 
an  infectious  nature.  Our  modern  knowledge  of  the  etiology  of  meningitis 
(see  above)  indicates  the  care  to  be  taken  of  traumatic  or  septic  affections. 

II.  TUBERCULAR  MENINGITIS 

This  form  of  meningitis  is  the  most  frequent.  It  is  due  toan  infection, 
of  the  meninges  by  Koch's  bacillus  which  has  a  special  predilection  for  the 
pia-mater.  In  the  majority  of  cases  it  occurs  in  very  young  children, 
especially  between  two  and  ten.  H.  Koch's  recent  investigations  (Ztschr. 
f.  Kinder heilk.,  v,  No.  5)  show  that  the  maximum  number  of  cases  occurred 
in  the  first  four  years  of  life  with  the  largest  number  in  the  second  year.  It 
rarely  occurs  after  thirty  years.  The  infection  is  always  secondary: 
it  develops  in  individuals  having  a  tubercular  focus  in  some  organ  or  tissue 
even  in  a  latent  state.  The  most  frequent  source  is  pleuro-pulmonary 
tuberculosis;  next  frequent  seat  is  found  in  the  mediastinal  and  mesenteric 
glands,  in  the  bones,  in  the  articulations,  in  the  intestines  and  in  the  genito- 
urinary tract.  The  manner  with  which  the  infection  is  produced  is  not 
settled.  It  is  generally  believed  that  the  bacilli  reach  the  meninges  through 
the  arterial  or  lymphatic  systems. 

In  considering  the  direct  cause  of  tubercular  meningitis  sight  should 
not  be  lost  of  the  predisposing  factors.  Tubercular  heredity  plays  an 
important  role.  Trauma,  unfavorable  hygienic  surroundings,  disturbances 
of  nutrition  due  to  some  prolonged  infectious  disease  (especially  measles 
and  whooping-cough),  prolonged  physical  exertion  and  mental  strain, 
alcoholism — are  all  causes  of  tubercular  meningitis  in  a  predisposed  indi- 
vidual. Tubercular  meningitis  may  develop  in  meninges  already  infected 
by  other  microbes.  Thus  a  purulent  meningitis  may  be  followed  by  a 
tubercular  meningitis.  Meningococcus  meningitis  may  terminate  by  a 
tubercular  meningitis  (Lion  and  LeBlaye,  Bull,  et  Mem.  Soc.  med.  des  hop. 
de  Paris,  19 10). 

Pathology. — The  characteristic  alterations  of  tubercular  meningitis 
are  predominant  at  the  base  of  the  brain.  They  are  found  principally  in 
the  area  of  circle  of  Willis,  between  the  chiasma  and  the  cerebral  peduncles, 
in  front  of  the  pons  and  around  the  medulla.  The  lesion  consists  of  (1) 
miliary  tubercles  and  (2)  common  inflammatory  products.  The  latter 
presents  a  serofibrinous  (rarely  purulent)  substance  which  may  extend 


1 86  DISEASES    OF   MEMBRANES    OE  BRAIN 

along  the  arteries  toward  the  convexity  of  the  brain;  it  covers  the  pia  and 
sends  out  prolongations  so  that  adhesions  are  formed  between  the  convolu- 
tions and  a  more  or  less  thick  layer  surrounds  the  branches  of  the  Sylvian 
artery,  the  chiasma  and  the  origin  of  the  cranial  nerves.  The  pia  itself  is 
congested,  cedematous  and  thickened. 

The  most  characteristic  formation  is  found  in  the  miliary  tubercles. 
Along  the  blood  vessels,  and  particularly  at  the  level  of  their  bifurcations 
are  seen  fine  grayish  nodules.  They  occupy  exclusively  the  pia.  These 
granulations  or  tubercles  may  adhere  to  each  other  and  form  groups. 
Sometimes  they  are  very  abundant  and  disseminated  over  the  entire  brain; 
but  habitually  they  are  localized  along  the  blood  vessels.  In  order  of 
frequency  they  are  found:  at  the  base  of  the  brain,  circle  of  Willis,  fissure 
of  Sylvius,  convexity  of  the  hemispheres,  finally  the  cerebellum,  pons  and 
medulla.  Histologically  they  consist  of  the  usual  tubercular  elements 
and  a  very  large  number  of  tubercle  bacilli. 

Besides  the  inflammation  of  the  meninges,  the  blood  vessels  and  the 
cortex  are  also  involved  in  tubercular  meningitis.  Periarteritis,  endar- 
teritis and  thrombosis  are  the  vascular  changes  usually  found  in  this 
affection.  The  above  mentioned  oedema  of  the  pia  as  well  as  the  changes 
in  the  brain  substance  are  due  to  them  to  a  great  extent.  The  extension 
of  the  meningeal  process  to  the  superficial  layer  of  the  cortex  will  produce 
encephalitis  (see  Encephalitis)  with  its  characteristic  condition,  viz. 
congestion  of  the  blood  vessels  and  oedema.  Softening  and  hemorrhagic 
foci  occur  in  the  deep  substance  of  the  brain;  they  are  the  result  of  oblitera- 
tion of  blood  vessels.     The  pia  is  intensely  adherent  to  the  cortex. 

Tubercular  meningitis  is  frequently  accompanied  by  abundant  exuda- 
tion in  the  ventricles  (hydrocephalus).  When  the  amount  of  fluid  is  con- 
siderable, the  ventricles,  being  distended,  compress  the  convolutions,  so 
that  complete  cessation  of  cerebral  functions  may  take  place,  viz.  coma 
and  death.  The  choroid  plexuses  are  thickened,  the  ependyma  is  softened 
and  torn  and  sometimes  tubercular  granulations  are  seen  on  it.  Tuber- 
culous meningitis  may  extend  to  the  membranes  of  the  spinal  cord.  We 
find  here  congestion  and  serous  or  sero-purulent  exsudates,  also  tubercular 
granulations.  The  latter  follow  the  course  of  the  blood  vessels.  The 
lesions  predominate  on  the  posterior  surface  of  the  cord  and  in  its  lumbar 
portion.  The  tubercular  infiltration  may  extend  to  the  nerve-roots  and 
spinal  ganglia. 

Finally  it  is  a  common  observation  to  find  tubercular  foci  in  viscera  or 
other  tissues,  and  particularly  in  the  lungs. 

Symptoms. — Three  periods  are  to  be  considered:  (a)  prodromal,  (b) 
period  of  cerebral  irritation,  (c)  period  of  depression. 


DISEASES    OF   MEMBRANES    OF  BRAIN  1 87 

Prodromal  symptoms  are  rarely  wanting,  especially  in  children.  The 
little  patient,  who  usually  suffers  from  some  latent  tubercular  focus, 
becomes  silent,  sad  or  unusually  irritable,  discontented;  he  loses  his  appe- 
tite, has  gastro-intestinal  disturbances,  especially  constipation,  complains 
of  vague  pains.  The  adult  becomes  languid  and  is  unable  to  do  mental 
work;  at  the  same  time  is  irritable  and  restless.  Then  insomnia  makes  its 
appearance  and  becomes  persistent.  This  is  soon  followed  by  headache 
and  occasional  vomiting.  The  child  is  pale,  loses  in  weight,  is  feverish. 
The  fever  is  usually  moderate.  The  pupils  are  unequal  and  dilated.  The 
pulse  is  irregular.  Photophobia  is  present.  Light  reflex  is  sluggish. 
The  reflexes  are  usually  exaggerated.  Convulsions  are  present;  they  are 
mostly  localized  and  especially  in  the  face  or  in  the  limbs.  Contractures 
are  characteristic  and  appear  early.  Kernig's  sign  is  not  frequent  at  the 
beginning,  but  Brudzinski's  sign  (see  above)  is  constant.  The  contrac- 
ture may  affect  the  muscles  of  the  eyes  and  produce  strabismus.  Retrac- 
tion of  the  abdomen  is  quite  constant.  Twitchings,  tremors,  involuntary 
movements  are  observed.  This  condition  may  last  from  several  weeks  to 
months.  The  transition  to  the  second  period  is  gradual.  Little  by  little 
the  above  symptoms  become  accentuated.  Particularly  three  symptoms 
become  conspicuous,  viz.  headache,  vomiting  and  constipation.  The 
temperature  is  usually  moderate  (ioo°-io2°)  and  of  a  remittent  character; 
its  chief  characteristic  is  the  irregularity:  sometimes  it  may  undergo  a 
transient  rise,  at  another  time  it  falls  much  below  normal.  The  pulse  is 
generally  unequal  and  irregular;  its  changes  are  not  parallel  with  those  of 
the  temperature:  the  latter  may  be  abnormal  by  its  rise,  the  former  by  its 
fall.  Toward  the  end  the  pulse  loses  its  irregularity'but  gains  in  rapidity. 
Respiration  is  also  irregular.  Deglutition  becomes  difficult  and  the  phar- 
yngeal reflex  is  lost.  All  these  manifestations  are  indicative  of  bulbar 
involvement  and  render  the  prognosis  unfavorable.  The  patient's  fades 
is  typical  by  its  immobility.  He  shows  impatience,  desire  to  be  let 
alone.  Gradually  delirium  enters  the  scene :  it  is  usually  mild,  with  slight 
agitation  and  interrupted'  occasionally  by  a  "hydrocephalic  cry."  (See 
Hydrocephalus.) 

Ocular  Symptoms. — Photophobia,  pupillary  inequality,  ophthalmo- 
plegia, nystagmus  and  oedema  of  the  fundus — are  all  constant  symptoms. 
They  are  due  to  the  basal  extension  of  the  pathological  process.  These 
cranial  nerve  symptoms  are  of  a  great  diagnostic  importance. 

The  duration  of  this  period  is  from  ten  to  fifteen  days.  Its  symptoms 
are  indicative  of  destruction  or  softening  of  brain  tissue  (see  Pathology). 
The  contractures  gradually  disappear  and  are  replaced  by  relaxation.  At 
this  stage  palsies  of  one  or  two  limbs  occur  and  they  usually  precede  the 


1 88  DISEASES    OF    MEMBRANES    OF  BRAIN 

generalized  relaxation.  Aphasia  is  not  rare.  Convulsions  are  rare  in  this 
phase.  The  patient  gradually  becomes  stuporous,  somnolent,  the  corneal 
reflex  disappears.  The  condition  may  last  several  days.  Coma  enters 
the  scene,  the  sphincters  become  relaxed,  respiration  difficult  and  of 
Cheyne- Stokes  type,  the  body  cyanosed.  The  temperature  after  a  rapid 
rise  falls  far  below  normal.     Death  is  inevitable. 

Course,  Termination  and  Prognosis. — Ordinarily  the  disease  runs 
a  sub-acute  course.  In  the  majority  of  cases  the  two  main  periods  of 
the  disease  last  two  or  three  weeks,  but  the  prodromal  phase  may 
last  from  one  to  several  weeks.  On  the  other  hand  remissions  present  a 
characteristic  feature  of  the  disease,  so  that  the  duration  may  be  prolonged. 
Death  is  the  natural  termination  in  most  of  the  cases,  if  not  in  all.  Rare 
cases  of  recovery  have  been  reported,  but  they  were  probably  of  non-tuber- 
cular nature,  or  cases  of  localized  tubercular  meningitis.  The  prognosis 
must  be  based  not  only  upon  the  general  symptomatology,  but  also  upon 
the  state  of  the  cerebro-spinal  fluid;  in  the  majority  of  cases  the  latter 
alone  will  decide  the  tubercular  or  non-tubercular  nature  of  the  menin- 
gitis. Tubercular  meningitis  in  children  and  in  adults  differs  from  each 
in  some  respects.  The  prodromal  period. is  frequently  absent  in  children. 
Instead  of  constipation  there  is  diarrhoea.  Convulsions  appear  early. 
The  fontanelle  is  bulging.  Rigidity  of  the  neck  and  contractures  in 
general  are  not  pronounced.  In  adults  mental  symptoms  are  conspicuous : 
delirium  is  frequent;  it  is  accompanied  by  a  confusional  state  and  some- 
times by  hallucinations.     Motor  symptoms  are  also  prominent. 

Diagnosis.— In  a  number  of  cases  the  diagnosis  presents  great  difficul- 
ties, as  on  one  hand  there  are  affections  (infectious  for  example)  in  the 
course  of  which  cerebral  symptoms  may  simulate  meningitis,  and  on  the 
other  hand  tubercular  meningitis  may  remain  latent  for  a  considerable 
time  and  present  at  the  beginning  only  a  few  vague  symptoms.  Hysteria 
sometimes  presents 'the  clinical  picture  of  meningitis,  including  the  fever. 
Similar  symptoms  may  be  observed  in  the  course  of  rachitis,  of  gastro- 
enteritis (in  infants),  of  influenza,  of  uremia,  of  hereditary  syphilis.  Al- 
though the  differential  symptoms  characteristic  of  each  of  these  diseases 
will  enable  us  to  recognize  them  in  the  majority  of  cases,  nevertheless  the 
diagnosis  is  not  always  possible  without  the  aid  of  a  new  procedure  which 
has  proven  to  be  of  great  utility.  I  speak  of  lumbar  puncture  (see 
chapter  on  Acute  Meningitis). 

The  following  are  the  characteristics  of  the  cerebro-spinal  fluid  of 
tubercular  meningitis.  Ordinarily  the  fluid  is  clear  but  at  close 
observation,  especially  when  it  is  looked  at  through  the  opening  of  the 
tube,  it  will  appear  slightly  greenish.     It  runs  out  under  high  pressure. 


DISEASES    OF   MEMBRANES    OF  BRAIN  1 89 

After  standing,  a  slight  coagulum  will  form.  The  albumen  content  is 
very  high  (1-2  grm.).  Sodium  chloride  in  normal  conditions  is  7  grm., 
43  per  hter.  In  tubercular  meningitis  its  contents  is  6  grm.,  36  (Nobecourt 
and  Voisin) . 

Normally  there  are  very  few  cellular  elements  in  this  fluid.  While,  as 
we  have  seen,  in  acute  meningitis,  the  polymorphonuclear  leucocytes  are 
abundant,  in  tubercular  meningitis  the  lymphocytes  predominate.  If 
polynuclear  leucocytes  are  met  together  with  the  lymphocytes,  the  former 
almost  always  show  degenerative  changes  (J.  Hohn,  Berl.  klin.  Wchn., 
1912).  Lymphocytosis  (160  per  cubic  millimeter)  exists  not  only  in  the 
typical  forms,  but  also  in  cases  with  a  latent  and  insidious  onset.  Recent 
researches  have  shown  that  lymphocytosis  is  the  expression  of  any  chronic 
morbid  process  of  the  meninges.  Thus  it  is  observed  also  in  tabes,  cerebro- 
spinal syphilis,  paresis,  and  multiple  sclerosis.  It  has  therefore  no  abso- 
lute diagnostic  value,  although  it  will  decide  with  absolute  certainty  the 
question  whether  a  meningeal  involvement  occurring  in  the  course  of 
other  diseases  is  of  a  tubercular  or  non-tubercular  nature. 

Another  interesting  peculiarity  of  the  cerebro-spinal  fluid  is  the 
permeability  of  the  meninges  for  certain  drugs,  for  example  for  iodide  of 
potash  or  methylene  blue.  Normally  the  pia-arachnoid  is  not  permeable 
from  outside.  Although  the  same  permeability  was  observed  in  uremia, 
nevertheless  this  phenomenon  is  absent  in  other  forms  of  meningitis  but 
present  in  the  tubercular.  When  a  patient  is  given  for  several  days  any 
of  these  two  drugs,  it  will  be  revealed  in  the  cerebro-spinal  fluid. 

As  to  the  specific  bacillus,  it  is  net  always  possible  to  find  it  in  the 
cerebro-spinal  fluid,  but  when  present,  the  diagnosis  is  established.  It 
is  absolutely  necessary  to  centrifugate  the  fluid  immediately  after  the 
lumbar  puncture,  because  the  fibrin  in  precipitation  englobes  in  its 
meshes  the  bacilli. 

In  every  case  of  suspected  tubercular  meningitis  the  cerebro-spinal  fluid 
should  be  examined  from  every  standpoint,  chemical  as  well  as  cyto- 
logical,  especially  in  cases  in  which  the  bacillus  cannot  be  found.  The 
above  mentioned  characteristics  of  the  fluid  are  not  absolute. 

Treatment. — There  is  no  specific  medication  for  tubercular  meningitis. 
The  treatment  is  purely  symptomatic.  Headache  will  be  relieved  by 
cold  applications  to  the  head;  antipyretics  will  be  used  for  fever,  sedatives 
and  narcotics  for  restlessness  and  insomnia,  frequent  warm  baths  for  con- 
tractures, purgatives  for  constipation.  Iodids,  also  the  usual  anti-tubercu- 
lar remedies,  as  creosote,  guaiacol,  etc.,  may  be  useful.  As  to  surgical 
means,  lumbar  puncture  may  relieve  cerebral  compression,  but  it  cannot 
be  considered  as  a  curative  measure.     A  permanent  drainage  of  the  cere- 


190  DISEASES    OF   MEMBRANES    OE  BRAIN 

bro-spinal  fluid  has  been  recommended,  but  it  is  a  procedure  associated 
with  some  risk. 

For  Haynes'  continuous  drainage  of  "Cisterna  Magna,"  see  page  184. 

III.  EPIDEMIC  CEREBRO-SPINAL  MENINGITIS 

Etiology. — The  epidemic  character  of  the  disease  is  very  important, 
as  it  is  sufficient  to  differentiate  it  from  other  forms  of  acute  meningitis. 
Sometimes  it  assumes  a  sporadic  form.  That  the  disease  is  contagious 
is  admitted  generally.  It  has  been  observed  that  the  disease  had  spread 
through  individuals  coming  from  localities  in  which  epidemic  meningitis 
existed. 

According  to  the  latest  investigations  the  etiological  factor  of  this 
disease  is  the  meningococcus  (diplococcus  intracellularis)  of  Weichselbaum. 

Cerebro-spinal  meningitis  may  develop  during  the  course  of  influenza 
and  be  due  exclusively  to  Pfeiffer's  bacillus,  but  this  is  not  the  genuine 
epidemic  form.  Sometimes  other  pathogenic  microbes  may  be  found  in 
the  cerebro-spinal  fluid  in  assoication  with  the  meningococcus,  such  as 
pneumococcus,  streptococcus,  etc.,  but  these  are  probably  cases  of  sec- 
ondary infection. 

Finally,  cerebro-spinal  meningitis  may  be  purulent  and  aseptic,  in 
which  no  bacilli  can  be  found  in  the  cerebro-spinal  fluid.  It  may  run  a 
similar  course  as  the  epidemic  form,  but  sero-therapy  has  an  unfavorable 
effect  on  it;  its  effect  on  the  leucocytic  contents  is  not  the  same  as  in  the 
epidemic  form. 

The  meningococcus  is  considered  pathognomonic  of  epidemic  cerebro- 
spinal meningitis,  as  statistical  studies  of  epidemics  have  shown  them  to 
be  present  in  eighty  per  cent.  Epidemic  cerebro-spinal  meningitis  is 
observed  at  all  ages  but  it  is  particularly  frequent  in  young  children. 
In  Koplik's  statistics  (Med.  News,  1904)  in  70  per  cent,  it  occurred  below 
the  age  of  four. 

The  disease  is  observed  more  in  winter  and  spring  than  in  other  seasons. 
The  epidemics  usually  do  not  last  longer  than  three  or  four  months  and 
develop  as  a  rule  slowly.  The  infection  is  transmitted  from  the  germ 
carrier  through  the  naso-pharyngeal  cavity  to  the  meninges,  very  probably 
via  the  blood  vessels,  but  this  question  has  not  been  entirely  settled. 

Pathology.— The  lesions  of  the  meninges  found  here  do  not  differ 
materially  from  those  found  in  other  forms  of  suppurative  meningitis. 
The  exsudate,  which  is  found  in  every  form  of  acute  meningitis  (see  this 
chapter),  is  here  purulent.  Pus  is  particularly  situated  along  the  veins 
and  predominates  at  the  base  of  the  brain  at  the  level  of  the  chiasma, 
in  the  region  of  the  fissure  of  Sylvius  and  around  the  cranial  nerves. 
The  ventricles  are  dilated  and  filled  with  a  cloudy  fluid.     The   choroid 


DISEASES    OF   MEMBRANES    OF  BRAIN  191 

plexuses  are  thickened  and  filled  with  pus.  The  ependyma  is  in  a  state  of 
inflammation.  In  the  spinal  cord  the  pus  is  especially  abundant  on  its 
posterior  surface  and  in  the  lumbar  region.  The  purulent  exsudate 
develops  sometimes  with  an  extraordinary  rapidity:  in  some  cases  twenty- 
four  and  even  five  hours  after  the  appearance  of  the  first  symptoms. 

The  dura-mater  of  the  brain  is  usually  not  affected,  but  is  frequently 
congested  in  the  cord.  The  brain  and  cord  are  usually  intact,  but  they 
may  also  undergo  softening  in  certain  areas.  Degenerative  changes  in 
the  cortical  cells,  also  in  the  cells  of  anterior  cornua  of  the  cord,  degenera- 
tion of  nerve  fibers  are  not  infrequently  observed.  The  spinal  roots 
and  ganglia  participate  in  the  pathological  process.  The  cerebro-spinal 
fluid  obtained  by  lumbar  puncture  presents  the  following  peculiarities: 

(1)  In  acute  stage.  The  fluid  is  cloudy.  The  sediment,  obtained 
either  by  standing  for  several  hours  or  by  centrifugation,  contains  numerous 
polynuclear  cells  which  are  distinctly  in  a  state  of  degeneration;  they  are 
deformed  and  stain  poorly.  Mononuclear  cells  and  lymphocytes  are  in 
a  small  number  (4  per  cent.).  Meningococci  are  found  in  the  degenerated 
polynuclear  cells.  Albumen  is  increased,  sodium  chlorid  diminished. 
Vincent's  precipitin  reaction  (see  page  183)  is  an  important  diagnostic 
element. 

(2)  In  the  period  of  amelioration,  especially  when  antimeningococcus 
serum  was  used,  a  rapid  improvement  in  the  appearance  and  contents  of 
the  fluid  is  seen.  It  becomes  clear  and  transparent.  Lymphocytosis 
takes  place  of  polynucleosis,  the  meningococci  disappear. 

The  blood  presents  a  marked  leucocytosis  reaching  sometimes  50,000. 
Meningococcus  may  be  found  in  cultures  of  blood. 

As  to  alterations  in  other  organs,  they  are  usually  those  found  in  the 
course  of  other  infectious  diseases:  exsudates  in  serous  cavities  (pleura, 
pericardium,  articulations),  hypertrophy  of  the  spleen,  etc. 

Symptoms. — In  the  majority  of  cases  the  onset  is  sudden  and  the 
symptoms  appear  in  the  midst  of  perfect  health.  In  some  cases  the  onset 
is  less  abrupt  and  is  preceded  by  a  few  prodromal  manifestations,  as  head- 
ache and  general  malaise.  At  all  events,  the  clinical  picture  at  the  begin- 
ning of  the  disease  is  almost  identical  in  all  forms  of  meningitis. 

Headache  is  never  absent  and  is  unusually  violent  and  tenacious. 
It  radiates  to  the  neck  and  along  the  spine.  The  latter  becomes  extremely 
tender  to  touch,  so  that  the  slightest  movement  of  the  body  gives  the  patient 
excruciating  pain.  The  entire  body  may  become  painful,  but  particularly 
the  joints.  Vomiting  is  always  present  and  is  of  cerebral  type  (see  Tumors 
of  Brain).  Chills  are  a  constant  initial  symptom.  These  three  symp- 
toms are  accompanied  by  a  rise  of  temperature,  as  high  as  1020.     Soon 


192  DISEASES    OF    MEiTBRAXES    OF   BRAIN 

contractures  make  their  appearance,  they  affect  particularly  the  muscles 
of  the  neck.  They  are  the  most  conspicuous  symptom  of  the  entire  clinical 
picture.  The  rigidity  of  the  neck  is  by  far  more  pronounced  than  in 
other  forms  of  meningitis.  The  head  may  be  drawn  back  almost  to  a 
right  angle.  When  the  muscles  of  the  back  are  affected,  the  body  may 
assume  the  form  of  opisthotonos.  Any  attempt  to  move  the  body 
increases  the  rigidity.  Kernig's  sign  (see  Acute  Meningitis)  is  almost 
always  present.  Brudzinski's  sign  (see  page  177)  is  constant.  Convul- 
sions are  frequent  in  children;  they  are  usually  generalized.  Sometimes 
they  are  followed  by  hemiplegia  or  palsies  of  cranial  nerves.  Tremors 
are  sometimes  observed.  The  knee-jerks  are  frequently  diminished  or 
abolished.  The  toe  phenomenon  is  frequently  present.  There  is  a  gen- 
eral hyperesthesia,  affecting  the  skin,  musculature  and  joints.  Vaso- 
motor disturbances  are  present:  alternating  redness  and  pallor  of  the 
face  are  common.  Taches  cerebrales  are  observed.  The  eye  symptoms 
consist  of  dilatation  or  contraction  and  inequality  of  the  pupils.  Strabis- 
mus and  diplopia  are  sometimes  present.  Suppuration  of  the  conjunctivae 
and  of  the  middle  ear  may  occur  sometimes;  the  meningococcus  and  the 
pneumococcus  have  been  found  in  the  pus. 

Herpes  on  the  face  and  on  the  lips  is  quite  frequent.  Erythema  of 
the  skin  is  seen  on  the  elbows,  knees  and  buttocks.  Purpuric  eruptions 
are  seen  in  very  grave  cases.  In  certain  epidemics  they  are  frequent,  so 
that  the  term  "spotted  fever"  is  applied  to  them.  In  purpuric  cases 
epistaxis  is  frequent;  The  pulse  and  respiration  are  very  irregular :  they 
vary  from  one  individual  to  another.  The  temperature  remains  elevated 
during  the  entire  course  of  the  affection. 

Delirium  and  agitation  are  generally  present  in  the  first  period  of  the 
disease,  especially  in  adults. 

When  the  patient  enters  the  second  phase  of  the  disease,  the  con- 
tractures and  convulsions  are  substituted  by  paralysis.  It  may  be  hemi- 
or  monoplegia,  or  else  paraplegia.  This  occurs  in  prolonged  cases  or  dur- 
ing convalescence.  In  cases  with  fatal  termination  the  agitation  is  re- 
placed by  depression  :  the  respiration  and  pulse  become  feeble,  the  delirium 
gives  place  to  coma.  In  the  fulminant  form  the  entire  course  of  the  dis- 
ease may  last  but  a  few  hours. 

Clinical  Forms. — Cerebro-spinal  meningitis  does  not  follow  the  same 
course  in  every  case.  The  acute  form  has  a  sudden  onset  with  chills,  vio- 
lent headache,  high  fever  and  vomiting.  The  characteristic  symptoms 
develop  rapidly,  convulsions  make  their  appearance  and  the  patient  usually 
dies  in  a  few  days.  In  the  so-called  fulminant  form  the  patient  becomes 
comatose  in  the  beginning  and  expires  at  the  end  of  but  a  few  hours.     The 


DISEASES    OF   MEMBRANES    OF   BRAIN  1 93 

protracted  form  is  characterized  by  remissions  and  phases  of  amelioration 
and  aggravation;  it  may  last  weeks  and  months.  In  case  of  recovery 
there  are  always  sequelae:  paralysis,  psychic  disturbances,  blindness  or 
deafness  and  in  infants  chronic  hydrocephalus.  Cases  have  been  also 
reported  in  which  the  patients  presented  mild  symptoms  and  were  not  bed- 
ridden ;  the  malaise,  Kernig's  sign,  rigidity  of  the  neck  and  lumbal  punctures 
made  the  diagnosis  unmistakable;  this  is  the  so-called  ambulatory  form. 

During  epidemics  an  abortive  form  of  cerebro-spinal  meningitis  is  some- 
times seen.  The  patient  feels  fatigued,  has  some  fever;  headache  and  rig- 
idity of  the  neck  develop.  All  these  symptoms  disappear  in  a  few  days. 
During  an  epidemic  all  manifestations  of  an  infectious  character,  no  matter 
how  mild  they  may  be,  should  be  looked  upon  with  suspicion.  In  such 
cases  the  meningococcus  should  be  investigated  not  only  in  the  cerebro- 
spinal fluid  but  also  in  the  naso-pharyngeal  cavity. 

Closely  connected  with  epidemic  cerebro-spinal  meningitis  is  the 
posterior  basic  meningitis  of  the  English  wi iters.  It  was  first  described 
by  Gee  and  Barlow  in  1878  and  the  organism  isolated  by  Still  in  1898. 
The  organism  resembles  greatly  Wechselbaum's  meningococcus.  The 
difference  lies  in  seme  cultural  characteristics.  The  most  striking  symp- 
toms are  according  to  the  English  writers:  early  head  retraction,  early 
blindness  and  subacute  course  of  the  disease.  Recovery  is  frequent,  but 
blindness  may  remain  permanent.  The  distinction  between  posterior 
basic  meningitis  and  the  epidemic  cerebro-spinal  meningitis  as  well  as 
the  precise  difference  between  Still's  and  Wechselbaum's  meningococci 
are  not  definitely  established. 

Prognosis. — It  depends  upon  the  form  of  the  affection  (see  above) 
and  upon  the  severity  of  the  epidemic.  Generally  speaking  it  is  grave, 
in  spite  of  sero-therapy.  It  is  especially  seiious  in  infants.  The  future  of 
the  patient  is  of  a  great  concern  in  view  of  the  complications  accompanying 
cerebro-spinal  meningitis  (sequelae) . 

Sequelae. — The  most  important  sequelae  are  those  showing  involvement 
of  the  nervous  system.  Encephalitis  and  myelitis  are  the  most  frequent. 
Paralysis  (hemiplegia,  monoplegia  or  paraplegia),  ataxia,  neuritis,  are 
very  common  complications.  Cranial  nerve  palsies  are  quite  frequent. 
Hydrocephaly  is  not  rare.  It  is  the  result  of  ependymitis  and  inflammation 
of  the  choroid  plexuses.  Otitis  interna  is  the  most  frequent:  deafness  is 
the  result  and  it  is  usually  bilateral.  Ocular  complications  are  frequent. 
They  are:  mydriasis,  inequality  of  pupils,  palsy  of  ocular  muscles,  iritis, 
papillitis  and  optic  atrophy.  Psychic  sequelae  are  not  rare.  They  are: 
change  of  disposition,  irritability,  diminution  of  intelligence. 

Diagnosis. — As  cerebro-spinal  symptoms  may  develop  in  the  course  of 

13 


194  DISEASES    OF   MEMBRANES    OE  BEAIN 

infectious  diseases,  the  diagnosis  sometimes  presents  difficulties.  There 
are  forms  of  cerebro-spinal  meningitis  caused  by  Pfeiffer's  bacillus  (in- 
fluenza) or  Eberth's  bacillus  (typhoid  fever).  The  character  of  the  cerebro- 
spinal fluid,  viz.  its  bacteriology,  chemistry  and  cytology  (see  above),  the 
character  of  the  onset,  the  course  of  the  affection  in  addition  to  the  exist- 
ence of  an  epidemic,  will  enable  to  make  a  correct  diagnosis.  For  differ- 
ential diagnosis  with  other  forms  of  meningitis  see  the  preceding  chapters. 

Tetanus  presents  trismus  at  the  beginning,  but  this  is  rare  in  cerebro- 
spinal meningitis.  Moreover  in  the  latter  affection  the  contractures  are 
much  more  easily  reducible  than  in  the  former. 

Hysteria  may  sometimes  simulate  the  disease,  especially  by  its  rigidity 
of  the  neck,  but  absence  of  fever  and  the  presence  of  special  stigmata 
in  addition  to  the  lack  of  other  symptoms  will  make  the  diagnosis  of  hys- 
teria easy. 

Uremia  will  be  differentiated  by  the  absence  of  fever,  herpes  and  other 
symptoms. 

Treatment. — The  general  principles  are  those  of  other  forms  of  menin- 
gitis. There  are  no  specific  medications.  The  symptomatic  treatment 
is  the  only  one  that  can  be  applied.  Sedatives  (bromides,  chloral,  mor- 
phin,  coal  tar  products)  are  useful  to  combat  insomnia  and  pain.  If 
the  heart  is  not  very  active  and  the  blood  pressure  not  very  high,  ergot 
given  hypodermatically  may  be  of  some  benefit.  An  adult  should  receive 
a  hypodermic  syringeful  (30  m.);  a  child  five  years  old,  one-fourth  of 
this  dose  (7  1/2  m.);  a  child  of  ten,  a  half  (15  m.).  Ergot  contracts  the 
blood  vessels,  relieves  congestion,  quiets  cerebral  excitement.  Hot  baths 
of  ten  to  twenty  minutes'  duration  repeated  every  three  or  four  hours 
according  to  patient's  general  condition  and  kept  up  during  the  entire 
course  of  the  disease  have  been  recommended.  Some  improvement  has 
been  also  obtained  from  saline  infusions.  Some  satisfactory  results  are 
being  reported  from  repeated  lumbar  punctures.  But  all  these  measures 
are  only  palliative. 

Sero-therapy  appears  to  be  the  only  means  of  curing  epidemic  cere- 
bro-spinal meningitis.  Flexner  and  Jobling  in  this  country  have  prepared 
an  antimeningococcus  serum  which  appears  to  give  excellent  results.  In 
Germany  Kolle  and  Wasserman,  in  France  Dopter,  and  in  Austria  Markl 
have  also  devised  sera  which  give  equally  good  results.  The  earlier  the 
serum  is  used  the  better  results  can  be  expected.  A  lumbar  puncture  is 
made  as  soon  as  the  disease  is  suspected,  about  30  c.c.  of  the  cerebro- 
spinal fluid  is  allowed  to  escape  and  an  equal  amount  of  serum  injected 
through  the  same  needle.  If  after  one  dose  improvement  is  noticeable 
no  further  injection  is  necessary.     In  the  majority  of  cases  improvement 


DISEASES    OF   MEMBRANES    OF  BRAIN  1 95 

is  noticed  after  the  first  injection.  Headache,  delirium,  insomnia,  fever, 
are  the  first  to  show  amelioration.  Rigidity  of  the  neck  and  other  con- 
tractures persist  longer.  The  cerebro-spinal  fluid  shows  progressive  im- 
provement: from  cloudy  it  becomes  clear:  the  polynuclear  cells  are  less 
altered,  the  lymphocytes  become  predominant;  albumen  decreases;  the 
precipi tin-reaction  (Vincent)  becomes  negative.  In  cases  in  which  the 
first  injection  is  not  followed  by  improvement,  a  larger  dose  of  serum 
should  be  given  daily  for  four  days.  After  the  four  injections,  wait  two 
or  three  days,  and  if  no  improvement,  resume  the  injections.  Flexner's 
statistics  since  the  use  of  his  serum  are  striking.  An  analysis  of  his 
523  cases  shows  a  mortality  of  25.4  per  cent.  In  Dopter's  cases  (339) 
the  mortality  was  11.80  per  cent.  The  German  statistics  show  a  mor- 
tality of  18.35  Per  cent.  Considering  the  tremendous  mortality  (80  per 
cent.)  before  the  introduction  of  the  serum,  the  latter  is  a  great  thera- 
peutic acquisition.  The  duration  of  the  disease  at  present  is  but  eight 
to  ten  days.  There  is  also  a  marked  diminution  of  the  sequelae.  The 
greatest  success  is  obtained  when  the  injections  are  made  at  the  earli- 
est possible  moment.  In  a  series  of  328  cases  Flexner  obtained  85  per 
cent,  recoveries  when  the  injection  was  made  from  first  to  the  third  day, 
78  per  cent,  when  it  was  made  from  the  fourth  to  the  seventh  day  and  61 
per  cent,  when  it  was  made  after  the  seventh  day.  Sero-therapy  does  not 
cure  every  case  of  meningitis.  It  fails  in  the  fulminant  cases,  in  cases 
of  associated  meningitis,  especially  in  tubercular  form,  finally  in  delayed 
cases.  In  grave  cases  Flexner  advises  two  injections  a  day.  The  same 
is  to  be  done  when  the  cerebro-spinal  fluid  remains  cloudy  and  contains 
meningococci.  As  to  the  dose,  10-15  c-c-  are  sufficeint  for  a  child  of 
two;  30  c.c.  in  an  older  child,  30-40  c.c.  in  an  adult.  Sometimes  the  dose 
must  be  increased.  Flexner  in  21  cases  had  to  use  200-300  c.c.  per  dose. 
Each  injection  should  be  preceded  by  an  evacuation  of  an  equal  or  rather 
greater  amount  of  cerebro-spinal  fluid.  The  lumbar  puncture  should  be 
made  in  the  fourth  space.  The  patient  is  placed  on  his  side  and  the 
dorsum  is  curved  as  much  as  possible.  As  soon  as  the injectionis  made,  the 
patient  is  placed  on  his  back,  the  head  is  lowered,  and  he  is  kept  in  this 
position  at  least  two  hours. 

Relapses  may  occur.  Of  1,294  patients  treated  by  Flexner,  56 
showed  relapses  after  a  greater  or  less  interval  of  time.  Prompt  resump- 
tion of  the  serum  injections  often,  but  not  invariably  sufficed  to  con- 
trol the  reinfections.  Of  the  56  patients,  40  recovered,  the  rest  died. 
Relapses  therefore  respond  less  well  to  the  serum  than  the  primary  infec- 
tions (/.  of  Experim.  Med.,  May,  1913).  The  same  author  observed 
that  complications  and  sequeke  are  favorably  influenced  by  the  serum. 


196  DISEASES    OF   MEMBRANES    OF   BRAIN 

The  least  influenced  is  deafness,  because  injury  to  the  internal  ear  takes 
place  very  early  and  sometimes  before  the  diagnosis  of  meningitis  has 
been  made.  Arthropathies  have  been  shown  to  be  amenable  to  direct 
injections  of  the  serum.  The  tendency  to  hydrocephalus  in  the  young 
has  been  diminished  and  the  intraventricular  injection  of  the  serum  in 
several  cases  controlled  the  infection  and  inflammation  of  cerebral  ven- 
tricles and  reestablished  communication  between  the  latter  and  the  sub- 
dural space  of  the  spinal  cord.     Recovery  has  been  rendered  complete. 

Sophian  (/.  Am.  Med.  Ass.,  191 3)  has  recently  formulated  special 
rules  for  a  safer  and  more  efficient  method  of  administration  of  antimenin- 
gococcus  serum.  From  an  extensive  experience  he  ascertained  that  the 
state  of  the  blood  pressure  is  an  excellent  guide  for  both  the  safe  with- 
drawal of  cerebro-spinal  fluid  and  injection  of  serum.  As  soon  as  the 
removal  of  fluid  commences,  there  is  a  drop  in  the  blood  pressure.  Con- 
sidering an  average  blood  pressure  of  no  mm.  of  mercury,  further  with- 
drawal of  fluid  should  stop  if  the  drop  of  blood  pressure  is  10  mm.  in  adults 
and  5  mm.  in  children.  The  serum  is  injected  by  gravity  method  better 
than  by  syringe.  The  entrance  of  serum  in  the  sub-arachnoid  space  is 
usually  followed  by  a  drop  of  blood  pressure  and  continues  to  drop. 
After  it  had  dropped  20  or  30  mm.  of  mercury,  the  further  dropping 
is  much  faster  if  more  serum  is  injected.  Sophian  finds  that  a  total  drop 
of  20  mm.  of  mercury  in  an  adult  with  average  blood  pressure  of  no  to 
120  mm.  of  mercury  is  a  safe  indication  to  stop  further  injection  of  serum. 
The  average  dose  of  serum  in  adults,  he  finds,  is  20  to  25  c.c.  and  it  is 
rarely  necessary  to  inject  more  than  40  c.c.  of  serum.  The  symptoms  of 
drop  in  blood  pressure  are:  stupor,  irregular  respiration,  slow  and  irregu- 
lar pulse,  dilatation  of  the  pupils,  incontinence  of  urine  and  feces.  In 
case  these  symptoms  become  pronounced,  as  much  fluid  as  possible  should 
be  removed  and  artificial  respiration  begun.  Intramuscular  injections  of 
epinephrin  and  atropin  should  be  administered. 

The  injections  of  serum  controlled  by  blood  pressure  have  given  Sophian 
more  satisfactory  results  than  with  the  old  method. 

The  immediate  object  of  antimeningococcus  serum  is  to  dissolve 
and  inhibit  the  multiplication  of  meningococci.  If  the  exsudate  in  the 
spinal  canal  is  thick,  the  diplococci  may  not  be  accessible.  To  render 
them  accessible  D.  I.  Hirsch  suggested  recently  (/.  Amer.  Med.  Assn., 
1 913)  irrigation  of  the  spinal  canal  before  injecting  the  antimeningococcus 
serum.  He  advises  the  preliminary  irrigation  in  every  case  of  cerebro- 
spinal meningitis.  He  removes  from  30  to  40  c.c.  of  fluid  by  lumbar 
puncture;  then  with  a  small  syringe,  using  gentle  pressure,  he  introduces 
30  c.c.  of  normal  salt  solution  ioo°  F.  into  the  canal;  he  allows  this  to 


DISEASE    OF    MEMBRANES    OF   BRAIN  197 

return;  he  repeats  this  three  times  in  those  cases  in  which  the  fluid  is 
cloudy,  and  in  purulent  cases  repeats  until  the  fluid  returns  clear.  Then 
only  he  injects  the  serum.  He  reports  two  cases  in  which  the  results  of 
this  treatment  were  highly  satisfactory. 

Serum  Accidents. — Sometimes  unpleasant  symptoms  follow  injections 
of  serum.  They  are:  skin  eruptions  of  the  type  of  urticaria;  rise  of  tem- 
perature; disturbance  of  respiration,  convulsions,  and  in  a  few  cases  a 
comatose  state.     All  these  complications  are  fortunately  rare. 

Prophylaxy  of  Cerebro-spinal  Meningitis. — Isolation  of  patients  and 
of  the  germ  carriers  when  found  and  disinfection  of  the  naso-pharyngeal 
cavity  and  of  all  the  surroundings  are  the  best  means  to  prevent  the 
spreading  of  the  disease. 

IV.  CHRONIC  MENINGITIS 

As  a  secondary  affection  chronic  meningitis  is  not  rare.  It  is  quite 
frequent  in  syphilis  of  the  nervous  system,  in  disseminated  cerebro- 
spinal sclerosis,  in  tumors  and  in  focal  lesions  of  the  brain,  also  as  sequelae 
of  acute  meningitis.     It  is  the  essential  lesion  of  paresis. 

There  is  also  a  primary  form  of  chronic  meningitis  (leptomeningitis). 
It  is  met  with  in  chronic  alcoholism  and  traumata.  Similar  to  pachy- 
meningitis (see  this  chapter),  it  affects  preferably  the  convexity  of  the 
brain.  The  pia-mater  is  thick  and  adherent  and  it  is  difficult  to  separate 
it  from  the  cortex.  At  the  base  it  occurs  very  exceptionally.  If  it  does, 
it  is  mostly  of  syphilitic  nature.  The  ventricular  ependyma  often  par- 
ticipates in  the  morbid  process,  particularly  in  the  fourth  ventricle: 
it  is   much   thickened   and   the   fluid  is   increased. 

Symptoms. — They  are  vague,  when  the  localization  is  on  the  convexity 
(in  alcoholism).  Diffuse  headache,  which  is  deep  and  very  severe,  also 
shooting  pain  along  the  course  of  cranial  nerves,  paralysis,  especially 
hemiplegic  form,  disturbance  of  speech,  papillary  oedema,  sometimes 
delirium,  finally  intellectual  enfeeblement,  constitute  the  whole  sympto- 
matology. When  the  base  is  involved,  there  are  also  palsies  of  cranial 
nerves.  The  chief  characteristic  of  chronic  meningitis  is  the  fact  that 
all  these  symptoms  are  not  encountered  in  combination  as  is  the  case 
in  acute  meningitis,  but  only  one  or  two  of  them  are  present  at  one  time 
and  become  fixed  and  persistent.  The  course  of  the  disease  is  very  slow 
and  death  is  the  rule. 

Diagnosis. — The  affection  may  be  confounded  with  cerebral  tumors 
and  cerebro-spinal  syphilis.     See  these  chapters. 

Treatment. — It  can  be  only  symptomatic. 


I98  DISEASE    OE   MEMBRANES    OE  BRAIN 

Aseptic  Cerebro-spinal  Meningitis. — Remlinger  (C.  R.  de  Soc.  de 
Biol.,  191 1)  investigated  this  form  of  meningitis  in  a  large  number  of 
soldiers  in  the  army.  Clinically  there  is  no  essential  difference  between 
it  and  the  epidemic  form,  except  that  the  prognosis  is  good.  Lumbar 
puncture  has  a  favorable  effect  on  the  disease.  Antimeningococcus 
serum  is  harmful.  The  cerebro-spinal  fluid  is  cloudy.  In  the  sediment 
obtained  from  centrifugation  are  seen  very  much  altered  polynuclear  cells. 
No  microorganisms  can  be  found.  Vincent's  precipito-reaction  (page 
183)  is  negative.  Cultures  also  remain  sterile.  The  disease  has  no  ep- 
demic  character:  the  cases  remain  isolated.  The  meningococcus  cannot 
be  found  in  naso-pharynx  or  in  any  other  part  of  the  body. 


CHAPTER  XIII 

THROMBOSIS  OF  THE  INTRACRANIAL  SINUSES 

For  the  anatomical  relations  of  the  sinuses  see  the  chapter  on  the 
Circulation  in  the  Anatomy  of  the  Brain. 

Pathology.- — The  thrombus  presents  a  grayish  or  reddish  clot  adherent 
to  the  walls  of  the  sinus.  It  may  affect  only  a  part  of  the  sinus  or  occupy 
its  entire  length  and  even  extend  into  the  tributary  veins.  Usually  the 
clot  does  not  entirely  fill  the  lumen  of  the  sinus.  The  resulting  venous 
stasis  produces  a  hyperemia  and  extravasation.  (Edema  is  considerable. 
The  cerebral  substance  becomes  softened.     The  ventricles  are  distended. 

The  phlebitis,  which  caused  the  thrombosis,  may  suppurate.  In 
such  cases  the  thrombus  is  purulent  and  metastases  are  then  often  seen  in 
various  organs;  cerebral  abscess  or  purulent  meningitis  may  also  follow. 

Etiology. — (a)  Primary  thrombosis  of  the  sinuses,  also  called  "marantic 
thrombosis,"  occurs  in  individuals  with  a  low  vitality,  as  in  tuberculosis, 
in  cancer,  in  chlorosis,  in  protracted  diarrhoeas  or  in  any  disease  of  long 
standing,  during  which  malnutrition  and  exhaustion  are  conspicuous.  It 
occurs  most  frequently  in  children  and  in  the  aged.  The  cause  of  throm- 
botic formation  lies  in  a  weakness  of  the  heart,  produced  by  the  above  dis- 
eases, and  therefore  in  retardation  of  circulation  and  increase  of  the  coagu- 
lability of  the  blood.     The  longitudinal  sinus  is  most  frequently  involved. 

(b)  Secondary  thrombosis  (inflammatory,  infective)  is  due  to  a  lesion 
in  the  neighborhood  of  the  dura.  The  inflammation  of  the  sinus  may  be  the 
result  of  the  direct  contact  with  the  diseased  area  or  may  occur  through 
the  veins  which  carry  the  infection  to  the  sinus.  Thus  thrombophlebitis 
follows  disease  of  the  skull  or  face  or  of  the  ear.  Caries  of  the  petrous 
bone  is  frequently  the  cause.  In  the  latter  case  the  sinuses  affected  are : 
lateral,  superior  and  inferior  petrosal.  Diseases  of  the  orbital  and  the 
nasal  cavities,  of  the  pharynx,  of  the  mouth,  periostitis  of  the  maxillary 
bones,  erysipelas  of  the  face,  are  all  causes  of  thrombophlebitis.  As  to  the 
microorganisms  in  the  thrombus,  nothing  definite  is  known:  strepto- 
coccus appears  to  be  the  most  frequent. 

Symptoms. — They  are  general  and  special.  The  general  symptoms 
are:  mental  hebetude,  somnolence,  headache  associated  with  vomiting, 
rigidity  of  the  neck  or  of  the  extremities,  sometimes  convulsions.  Optic 
neuritis  is  frequently  present,  especially  in  cases  of  chlorosis.  In  secondary 
or  infective  thrombosis  there  are  also  rigors  followed  by  profuse  perspira- 

199 


200 


THROMBOSIS    OF   INTRACRANIAL    SINUSES 


tion  and  elevation  of  temperature;  oedema  in  the  vicinity  of  the  sinus; 
pain  in  the  abdomen,  diarrhoea  may  be  present  in  some  cases;  great 
restlessness  with  delirium  may  be  present  in  others.  The  special  symp- 
toms depend  upon  the  sinus  involved. 

i.  Longitudinal  sinus:  Distention  of  the  temporal  veins;  cyanosis 
of  the  face;  tension  of  the  large  fontanelle  in  children;  epistaxis. 

2.  Transverse  sinus:  The  internal 
and  external  jugular  veins  are  empty. 
The  mastoid  region  is  cedematous  and 
painful. 

3.  Cavernous  sinus:  Stasis  and 
cedema  in  the  ophthalmic  vein.  (Edema 
of  the  orbit  and  of  the  eyelids.  Con- 
gestion and  cedema  of  the  retinal  blood 
vessels.  Exophthalmos.  Impairment 
of  vision.  Paralysis  of  the  third  and 
sixth  nerves,  also  of  first  branch  of  the 
fifth  nerve.  The  accompanying  photo- 
graph is  taken  from  a  patient  seen  with 
Dr.  Gittelson  at  the  Mount  Sinai  Hcs- 

Fig.  77  — Thrombosis  of  Right  Caver-  pital,  who    suffered  from  a  thrombosis 
nous  Sinus.     (Edema  of  Right  Side  of  0f  the  right  Cavernous  sinus.      Autopsy 

the  Face.     Exophthalmos  of  the  Right  -r    j  ,i       j- 

Eye  verified  the  diagnosis. 

Eye  symptoms  in  sinus  thrombosis 
have  sometimes  a  diagnostic  value.  The  following  are  the  most  impor- 
tant ocular  manifestations. 

(a)  Marantic  form  of  thrombosis.  Here  the  eye  disturbances  are  not 
as  frequent  as  in  the  infective  form. 

Venous  stasis  and  hyperemia  in  the  fundi,  optic  neuritis,  fixed  pupil 
(stauungspapilla) — are  all  occasionally  met  with.  More  frequently 
are  met  disturbances  in  ocular  movements,  particularly  conjugate  devia- 
tion of  the  eyes.  In  such  cases  both  sinuses,  longitudinal  and  trans- 
verse, are  simultaneously  involved.  The  third  nerve  is  oftener  affected 
than  others.  Exophthalmos  is  rare,  and  if  it  does  occur,  there  is  an 
involvement  of  the  cavernous  sinus  and  of  the  ophthalmic  vein. 

(b)  Infective  Thrombosis. — Apart  frcm  otitic  and  traumatic  cases, 
eye  symptoms  are  very  frequent  in  septic  thrombosis.  According  to 
Uhthoff — hyperemia  of  the  papillae,  optic  neuritis,  fixed  pupils,  optic 
atrophy — occur  in  20  per  cent,  of  cases.  The  involvement  of  cavernous 
sinus  in  septic  sinus  thrombosis  occurs  in  80  per  cent.  This  explains  the 
frequency   of   eye    disturbances.     Exophthalmos   is    the   most   frequent 


THROMBOSIS    OF   INTRACRANIAL    SINUSES  201 

symptom.  Uhthoff  observed  it  in  72  per  cent,  of  cases.  When  the 
sphenoid  bone  is  affected  with  secondary  septic  thrombosis  of  the  caver- 
nous sinus,  the  exophthalmos  is  bilateral.  Palsy  of  ocular  muscles  is 
almost  a  rule  when  the  cavernous  sinus  is  involved.  In  sinus  thrombosis 
of  otitic  origin  visual  disturbances  and  changes  in  the  fundi  are  of  great 
diagnostic  value.  The  latter  are  frequently  bilateral  like  in  other  intra- 
cranial affections.  Immobility  of  pupils  develops  quite  rapidly.  Jansen 
found  fixed  pupils  in  30-40  per  cent,  of  cases.  Optic  atrophy  according 
to  Uhthoff  is  found  in  24  per  cent,  of  cases.  Exophthalmos  is  by  far  less 
frequent  in  thrombosis  of  otitic  origin  than  in  other  septic  forms  of  throm- 
bosis.    Palsy  of  ocular  muscles  occurs  when  the  cavernous  sinus  is  involved. 

Duration.  Termination.  Prognosis. — The  secondary  thrombo-phlebi- 
tis  has  a  different  course  from  the  primary  (marantic).  The  sudden 
onset,  the  rigor,  the  violent  headache  and  the  very  high  temperature  are 
indicative  of  a  purulent  state.  Its  duration  is  usually  a  few  days.  It 
may  be  complicated  by  an  abscess  in  the  temporo-sphenoidal  lobe.  The 
marantic  form  is  more  insidious  in  onset  and  longer  in  duration.  Death 
is  the  usual  termination. 

Diagnosis.- — Appearance  of  cerebral  symptoms  in  an  individual 
suffering  from  any  of  the  diseases  mentioned  in  etiology  should  arouse 
suspicion  in  regard  to  a  phlebitis  and  obstruction  of  the  sinuses.  How- 
ever the  symptoms  may  simulate  meningitis,  but  the  special  signs  char- 
acteristic  of  each  sinus-obstruction  will   enable   to   reveal   thrombosis. 

The  following  features  will  enable  one  to  distinguish  between  a  marantic 
and  an  infective  thrombosis.  The  usual  seat  of  the  first  is  the  single  sinuses, 
such  as  the  longitudinal;  the  seat  of  the  second  is  in  those  that  are  bilateral. 
The  first  has  a  tendency  to  become  organized  and  absorbed;  the  second, 
to  suppuration.  In  the  course  of  the  first  cerebral  hemorrhages  occur; 
in  the  second  hemorrhages  are  rare.  The  first  has  a  tendency  to  softening 
which  is  not  the  case  in  the  second.  Septic  metastatic  infections  are  fre- 
quent in  the  second,  rare  in  the  first.  Purulent  meningitis,  cerebral 
abscess,  are  common  in  the  second,  but  absent  in  the  first  (Macewen). 

Treatment. — Prophylactic  measures  are  the  most  important.  The 
most  rigorous  attention  should  be  given  to  diseases  of  the  ear  and  others 
to  prevent  secondary  infection.  The  medical  treatment  of  thrombosis 
will  be  purely  symptomatic.  In  the  marantic  form  tonifying  measures 
are  necessary.  The  original  disease  must  be  treated  most  energetically- 
Saline  infusions  copiously  given  have  given  me  satisfactory  results.  The 
surgical  treatment  is  advisable  only  in  the  secondary  form  of  thrombo. 
phlebitis.  Opening  and  curetting  of  the  affected  sinus  may  sometimes 
be  of  benefit. 


CHAPTER  XIV 

CIRCULATORY  DISTURBANCES  OF  THE  BRAIN 

ANEMIA  AND  HYPEREMIA 
Anemia  (Generalized) 

Pathology. — The  meninges  and  the  cortical  substance  are  pale.  Some- 
times cedema  with  effusion  between  the  convolutions  is  observed. 

Etiology. — It  occurs  most  frequently  in  the  two  extreme  ages  of  life. 
Hemorrhages  more  or  less  abundant;  rapid  evacuation  of  pleuritic  or 
ascitic  fluid;  cardiac  diseases,  diseases  of  the  blood;  chlorosis,  pernicious 
anemia,  leukemia;  inanition;  intracranial  pressure  (tumors,  hydrocephalus) ; 
embolism  or  thrombosis  of  cerebral  arteries;  lead  or  nicotin  intoxications 
— these  are  the  usual  causes  of  cerebral  anemia  in  adults.  Finally  vascular 
constriction  leading  to  anemia  may  be  caused  by  intense  emotion  and  by 
certain  drags,  viz.  ergot,  belladonna,  chloroform,  etc. 

Symptoms. — They  are  different  in  the  acute  and  chronic  forms. 

Acute. — In  anemia  of  a  sudden  onset  the  patient  becomes  dizzy.  This 
is  accompanied  by  a  noise  in  the  ear  and  disturbance  of  vision.  Vomit- 
ing is  quite  frequent.  Convulsive  movements  are  not  rare.  The  heart 
is  slow.  Arterial  pressure  is  increased.  An  imperative  somnolence  sets 
in  early  and  coma  may  follow;  pupils  then  become  dilated,  the  reflexes 
are  lost,  the  respiration  is  difficult.  The  blood  pressure  falls,  the  heart 
becomes  accelerated.  Death  occurs  soon.  If  the  cerebral  circulation  is 
reestablished,  the  above  symptoms  rapidly  disappear.  In  milder  forms 
convulsions  and  vomiting  are  absent.  In  exceptional  cases  optic  atrophy 
has  been  observed. 

Chronic. — This  variety  is  met  with  mainly  in  chlorotic  patients.  The 
psychic  symptoms  are  the  most  conspicuous.  Headache,  deficient  mem- 
ory, difficulty  of  mental  application,  amnesia,  irritability  or  else  marked 
depression  are  frequently  observed.  Insomnia,  vertigo,  occasionally  hal- 
lucinations and  delirium  are  also  met  with  in  chronic  cerebral  anemia. 

In  young  children  Marshall  HaU  observed  a  form  of  cerebral  anemia 
to  which  he  gave  the  name  "hydrocephaloid"  from  its  resemblance  to 
hydrocephalus.  It  occurs  mostly  in  exhausting  diarrhoeas.  This  infan- 
tile anemia  has  a  period  of  agitation  followed  by  a  period  of  depression. 
The  little  patient  is  pale  and  apathetic;  the  pupillary  light  reflex  is  lost;  the 
pulse  and  respiration  are  irregular;  death  occurs  in  coma. 

202 


CIRCULATORY  DISTURBANCES    OF  BRAIN  203 

In  old  individuals  cerebral  anemia  is  the  result  of  an  atheromatous 
condition  of  the  blood  vessels.  Headache,  vertigo,  confusion  are  the 
main  symptoms. 

Prognosis. — It  is  favorable  in  mild  forms.  Convulsions  are  a  grave 
omen.     Speaking  generally,  the  prognosis  depends  upon  the  cause. 

Diagnosis. — As  the  symptoms  of  cerebral  anemia  and  hyperemia 
are  similar,  the  diagnosis  presents  in  this  respect  great  difficulties.  Potain's 
rule  will  enable  to  differentiate;  in  hyperemia  lowering  of  the  head  will 
produce  vertigo;  in  anemia  raising  of  the  head  will  cause  it. 

Treatment. — In  the  acute  form  the  horizontal  position  of  the  head  is 
the  first  indication.  The  increase  of  blood  in  the  head  can  be  aided  by 
placing  a  binder  on  the  lower  extremities.  Cardiac  stimulants,  especially 
alcohol,  should  be  resorted  to.  Artificial  respiration  and  saline  infusion 
are  beneficial.  In  the  chronic  form  the  treatment  will  be  that  of  vascular 
diseases  or  of  anemia. 

Hyperemia 

Pathology. — The  blood  vessels  and  the  sinuses  are  overfilled  with 
blood.  The  ventricles  and  sub-arachnoid  spaces  present  an  increase  of 
cerebro-spinal  fluid.  The  cortex  and  white  matter  are  reddish.  In  per- 
sistent passive  hyperemia  changes  in  the  cortical  cells  and  especially  in 
Purkinje's  cells  have  been  observed. 

Etiology. — Hyperemia  may  be  active  and  passive. 

Active  cerebral  congestion  may  be  due  to  any  cause  which  produces 
vaso-dilatation  of  cerebral  vessels.  Infectious  diseases,  insolation,  alcohol 
and  other  intoxications,  organic  cerebral  affections  are  the  usual  causes  of 
active  cerebral  congestion.  Sudden  contraction  of  the  peripheral  vessels, 
the  transition  of  sleep  to  the  waking  state,  a  physical  or  mental  effort, 
sensory  excitement  which  leads  to  a  rise  of  arterial  pressure,  are  also 
accompanied  by  cerebral  hyperemia. 

Passive  congestion  occurs  mostly  in  cardiac  diseases  during  the  period 
of  asystoly;  in  chronic  pulmonary  diseases,  in  emphysema.  Finally 
tumors  or  any  mechanical  obstruction  compressing  the  jugular  veins 
or  the  abdominal  aorta  will  also  produce  a  passive  congestion  of  the  brain. 

Symptoms. — In  active  congestion  the  initial  symptoms  may  be  acute 
and  subacute.  In  the  acute  form  the  onset  is  very  sudden  and  loss  of 
consciousness  may  occur.  Fortunately  the  symptoms  usually  improve 
rapidly,  but  a  transient  aphasia  or  hemiplegia  may  fellow.  Sometimes 
epileptiform  convulsions  with  or  without  loss  of  consciousness  may 
accompany  an  acute  attack.  In  some  cases  delirium jmay  be  the  initial 
symptom. 


204  CIRCULATORY  DISTURBANCES    OF  BRAIN 

In  the  subacute  form  the  patient  feels  a  rush  of  heat  to  the  head;  sees  a 
peculiar  flickering  befcre  his  eyes;  has  a  throbbing  in  his  temples,  has 
headache,  is  apathetic  and  somnolent.  The  pulse  is  small,  the  facies  is 
cyanosed,  the  conjunctivae  are  injected. 

The  passive  cerebral  congestion  is  essentially  chronic.  Headache, 
persistent  insomnia,  vertigo,  depression,  finally  turgid  face — are  the  usual 
symptoms. 

Diagnosis. — The  transient  character  of  the  paralytic  attacks  of  the 
grave  form  of  acute  cerebral  congestion  will  enable  to  differentiate  from 
similar  attacks  in  apoplexy,  in  which  the  history  of  the  case  and  the 
course  decide  the  diagnosis.  The  same  can  be  said  of  the  epileptiform 
seizures.  From  cerebral  anemia  it  will  be  differentiated  by  Potain's  rule 
(see  Anemia) . 

Prognosis. — It  is  usually  favorable,  if  the  condition  is  not  due  to  a 
cardiac  affection.     If  the  heart  is  diseased,  there  is  always  danger. 

Treatment. — Removal  of  the  cause  is  the  first  indication;  consequently 
prophylactic  measures  for  preventing  excesses  or  other  causes  leading  to 
congestion  are  the  most  important. 

In  an  attack  the  following  means  should  be  adopted.  Bleeding, 
leeching,  blistering,  purging  are  well-known  measures.  The  head  must 
be  raised.  Ice  applied  to  the  head  gives  relief.  Hot  foot  baths  are  valu- 
able. Internally  bromides  are  advisable,  but  opiates  and  chloral  should 
be  avoided.  In  passive  congestion  removal  of  tumors  or  of  other  mechan- 
ical obstructions  is  directly  indicated. 


CHAPTER  XV 
FRACTURES  OF  THE  SKULL 

The  study  of  this  subject  is  very  important.  As  it  frequently  happens, 
the  intracranial  contents  are  almost  invariably  involved  in  fractures  of  the 
skull.  The  knowledge  of  the  seat  of  the  injury,  of  its  character,  of  its  pos- 
sibilities, of  the  degree  of  involvement  of  the  intracranial  tissue  and  of  the 
seat  of  the  latter  will  enable  one  to  make  a  prompt  decision  as  to  immediate 
or  remote  surgical  intervention. 

Fractures  may  be  incomplete  or  ccmplete.  In  the  incomplete  variety 
there  may  be  either  a  fracture  of  the  outer  or  of  the  inner  table  of  the  skull. 
The  former  is  observed  chiefly  in  regions  where  the  two  tables  are  separated, 
like  the  frontal  sinus.  Isolated  fractures  of  the  inner  table  are  rare. 
Complete  fractures  affecting  both  tables  simultaneously  are  not  rare. 
In  this  case  the  fracture  may  be  only  a  fissure,  linear  or  else  present  sepa- 
rate fragments. 

Another  important  variety  is  the  radiating  one.  This  form  is  the  most 
frequent  in  fractures  of  the  base  of  the  skull.  The  radiation  towards  the 
base  is  done  by  the  shortest  possible  route.  A  fall  on  the  frontal  region  will 
produce  a  fracture  which  will  reach  the  anterior  portion  of  the  base.  In 
a  fall  on  the  temporal  region  the  fracture  will  reach  the  middle  portion  of 
the  base.  A  fracture  in  the  occipital  region  will  reach  the  posterior  portion 
of  the  base.  The  most  frequent  of  all  is  the  fracture  of  the  middle  portion 
of  the  base.  The  reason  of  it  lies  first  in  the  frequency  of  falls  on  the 
temporal  region  and  second  in  the  thinness  of  the  cranial  wall  at  this  level. 
The  fracture  reaches  the  middle  cerebral  fossa  and  ends  either  in  the  sphe- 
noid bone  or  in  the  petrous  bone.  In  the  latter  case  very  important  ele- 
ments may  be  involved,  viz.  the  external  auditory  meatus,  the  internal 
ear,  Fallopian  canal,  internal  auditory  meatus.  The  fracture  may  spread 
either  forward  on  the  same  side  and  involve  the  chiasma  or  reach  the  other 
side,  or  it  may  extend  posteriorly  to  the  cerebellar  region.  An  important 
variety  of  fractures  is  the  one  par  contre-coup,  Here  the  lesion  lies  in  an- 
other portion  of  the  skull  than  in  the  one  that  was  injured.  Its  localiza- 
tion is  frequently  either  the  occipital  region  or  the  petrous  bone,  but  most 
frequently  the  orbital  region. 

Fractures  of  the  skull  are  frequently  followed  by  involvement  of  the 
membranes,  brain  itself  and  cranial  nerves  at  the  base.     The  dura  is  either 

205 


206  FRACTURES    OF   THE    SKULL 

detached  or  torn.  The  blood  vessels,  the  sinuses,  may  also  be  torn,  hence 
extra-  or  intra-dural  hematoma.  If  the  arachnoid  suffers,  the  cerebro- 
spinal fluid  will  find  its  way  through  the  opening  in  the  skull,  or  through 
the  nasal  fossae  or  else  through  the  external  auditory  meatus.  A  tear  in 
the  pia  will  produce  a  hemorrhage.  Contusion  of  the  brain  is  constant. 
The  cranial  nerves  at  the  base  are  frequently  involved. 

Symptoms. — When  the  fracture  is  open,  besides  the  fragments  or  de- 
pression of  the  bones  we  may  notice  cerebro-spinal  fluid  or  brain  tissue 
oozing  through  the  opening.  When  the  fracture  is  closed,  the  chief  symp- 
toms are:  swelling  of  the  scalp,  depression  of  the  bone  and  pain.  As  to 
recognition  of  depressions,  one  must  always  bear  in  mind  a  natural  con- 
genital anomaly  of  the  skull  or  else  a  deformity  caused  by  a  previous  in- 
jury; in  such  a  case  there  will  be  no  swelling  of  the  scalp.  Hematoma  of 
the  scalp  may  sometimes  give  the  impression  of  a  fracture. 

In  fractures  radiating  towards  the  base,  the  following  symptom  is  most 
pathognomonic :  bleeding  from  the  nose,  ear  and  mouth.  Nasal  bleeding 
may  of  course  originate  in  the  nose  itself  in  case  the  latter  is  fractured.  In 
such  a  case  the  diagnosis  will  be  made  from  pain  and  swelling  in  the  nasal 
region,  also  crepitation  of  the  nasal  bones.  Bleeding  from  the  mouth  is 
the  result  of  fracture  of  the  sphenoid  or  of  the  roof  of  the  pharynx.  The 
bleeding  from  the  ear  is  the  result  of  fracture  of  the  petrous  bone  (see 
Anatomy  above).  A  bleeding  from  the  ear  may  be  due  to  a  tear  in  the 
tympanic  membrane,  but  then  the  bleeding  is  slight  and  very  brief. 
Besides,  in  the  latter  case  the  disturbance  of  hearing  is  much  less  marked 
than  in  fractures  of  the  petrous  bone.  Moreover  in  basal  fractures  we  find 
facial  palsy  and  total  deafness.  Sometimes  the  cerebro-spinal  fluid  finds 
its  way  through  the  ear,  more  rarely  through  the  nose.  In  basal  fractures 
ecchymoses  of  the  conjunctivae,  the  eyelids,  the  mastoid  and  the  neck  may 
occur.  The  first  is  the  most  frequent.  It  is  due  to  infiltration  of  the  cell- 
ular tissue  of  the  orbit  with  blood  originating  in  a  fractured  focus  of  the 
anterior  portion  of  the  base. 

A  very  important  diagnostic  point  lies  in  the  presence  of  blood  in  the 
cerebro-spinal  fluid,  and  in  the  high  tension  under  which  it  appears  upon 
lumbar  puncture.  In  order  to  avoid  errors,  it  is  advisable  to  collect  the 
fluid  in  three  successive  tubes.  If  only  the  first  tube  is  colored,then  the 
blood  comes  from  a  blood  vessel  during  the  lumbar  puncture.  If  the  col- 
oration is  the  same  in  all  the  three  tubes,  the  blood  is  in  the  cerebro-spinal 
fluid.  The  blood  is  due  to  its  entrance  from  the  torn  blood  vessels  into  the 
sub-arachnoidal  spaces. 

In  addition  to  these  physical  signs  there  are  also  functional  disturbances. 

First  of  all  the  shock  more  or  less  marked  immediately  after  the  injury. 


FRACTURES    OF    THE    SKULL  207 

Then  the  cerebral  manifestations  which  depend  upon  the  area  involved 
and  which  may  appear  either  immediately  or  some  time  later.  Thus  we 
may  have  epileptiform  convulsions,  disturbances  of  sensations  and  paralytic 
phenomena,  such  as  monoplegia,  hemiplegia  or  paraplegia  (see  Cerebral 
Localizations).  Paralysis  of  cranial  nerves  will  give  valuable  data  for 
localization.  The  most  frequent  occurrence  in  fractures  of  the  base  is 
paralysis  of  the  seventh  and  eighth  nerves. 

When  the  facial  palsy  follows  immediately  after  the  accident,  it  is  an 
indication  that  the  nerve  is  completely  torn  within  the  Fallopian  canal. 
If  the  facial  palsy  appears  several  days  after  the  fracture,  it  is  due  either 
to  compression  by  the  extra vasated  blood,  or  to  the  newly  forming  callus 
or  else  to  a  secondary  periostitis.  In  these  cases  the  facial  palsy  is  curable. 
The  palsy  is  of  the  peripheral  type. 

The  auditory  nerve  may  be  totally  injured.  The  involved  cochlear 
branch  -produces  impairment  of  hearing  or  deafness,  while  the  injured 
vestibular  branch  causes  vertigo,  loss  of  equilibrium  and  tinnitus. 

Paralysis  of  nerves  supplying  the  ocular  muscles  is  not  rare  in  fractures 
of  the  skull  especially  at  the  base.  The  most  frequent  is  palsy  of  the 
sixth  nerve.  It  follows  fracture  of  the  apex  of  the  petrous  bone.  The 
symptoms  are:  internal  strabismus  with  diplopia.  Paralysis  of  the  third 
nerve  is  exceptional. 

Prognosis. — Fractures  of  skull  are  always  a  serious  condition,  not  only 
on  account  of  immediate  but  also  of  remote  consequences.  Speaking 
generally  fractures  of  the  vertex  are  less  serious  than  those  of  the  base. 
The  outlook  depends  upon  the  intensity  of  nervous  phenomena,  vas- 
cular lesions,  pressure  of  cerebro-spinal  fluid  during  a  lumbar  puncture, 
infectious  symptoms  of  meningo-encephalitis,  sinus  phlebitis.  The  most 
important  factor  which  determines  the  gravity  of  any  given  case  is  the 
hemorrhage :  the  more  abundant  the  latter  is,  the  more  serious  is  the  out- 
look. When  the  fracture  runs  a  favorable  course,  it  usually  ends  in  form- 
ation of  a  callus  or  when  the  separation  of  the  fragments  is  considerable, 
a  newly  formed  fibrous  membrane  takes  the  place  of  the  callus. 

Treatment. — It  is  the  domain  of  the  surgeon  to  take  special  care  of  the 
wound,  to  avoid  secondary  infection  by  means  of  most  rigorous  aseptic  and 
antiseptic  measures.  In  case  of  localized  symptoms,  such  as  paralysis, 
convulsions  cr  sensory  disturbances,  an  operative  intervention  is  indicated. 
In  cases  of  depression  of  the  skull  even  without  immediate  symptoms,  an 
operation  should  be  undertaken,  because  cerebral  symptoms  of  paralytic  or 
convulsive  nature  may  develop  later.  As  to  the  nature  of  the  operation, 
large  osteoplastic  flaps  give  the  most  satisfactory  field  for  examination  of 
the  underlying  soft  tissue  and  for  a  more  complete  removal  of  the  accumu- 


2C>8  FRACTURES  OF  THE  SKULL 

lated  blood.  In  cases  in  which  localized  manifestations  are  on  the  same 
side  as  the  fracture,  it  is  advisable  first  to  operate  at  the  seat  of  the  frac- 
ture. If  the  results  are  not  satisfactory,  operate  on  the  opposite  side  of 
the  brain. 

In  fractures  of  the  base  which  is  not  accessible,  Cushing  obtained 
encouraging  results  from  decompressive  operations  in  the  subtemporal 
region  (Ann.  of  Surgery,  1908). 

Recently  lumbar  puncture  has  been  recommended  in  cases  of  fractures 
at  the  base  of  the  skull.  The  diagnostic  value  of  lumbar  puncture  has 
been  mentioned  above.  Very  favorable  results  have  been  reported  in 
traumatic  cases  treated  by  puncture  of  the  spinal  canal  and  extraction  of 
certain  amounts  of  the  cerebro-spinal  fluid  (Lenhartz,  Meslier,  Muret, 
Quenu,    Fowler   and   others).     The  advantage,    of  this    procedure  are: 

(1)  relieve  intracranial  pressure  and  through  it  the  cerebral  phenomena. 

(2)  remove  partly  the  microorganisms  which  are  the  cause  of  secondary 
meningeal  manifestations;  blood  is  an  excellent  medium  for  development 
of  germs. 

In  cases  of  injury  to  the  head  without  an  evident  fracture  of  the  skull, 
expectant  treatment  is  most  advisable.  The  patient  is  kept  quietly  in 
bed  and  watched  very  closely.  At  the  appearance  of  localized  or  other 
disturbances,  the  therapeutic  conduct  is  promptly  to  be  decided  upon. 


CHAPTER  XVI 
CONCUSSION  OF  THE  BRAIN 

The  subject  of  concussion  of  the  brain  is  of  practical  importance.  The 
common  belief  that  only  in  injuries  accompanied  by  loss  of  consciousness 
there  is  concussion  of  the  brain,  is  erroneous.  There  are  many  traumatic 
cases  in  which  there  is  no  genuine  loss  of  consciousness,  but  only  a  vertigo, 
or  a  transient  mental  hebetude,  or  else  a  hazy  recollection  of  the  trauma — 
all  cases  in  which  symptoms  of  a  grave  nature  may  develop  immediately 
or  some  time  after  the  accident.  An  appreciation  of  these  milder  forms 
of  cerebral  concussion  is  of  importance  from  a  therapeutic  standpoint,  as 
if  they  are  ignored  or  overlooked,  serious  consequences  may  follow. 

Pathogenesis. — The  nature  of  concussion  is  still  debatable.  As 
autopsies  are  exceedingly  rare,  a  pathogenesis  cannot  be  established  with 
any  degree  of  precision.     The  following  views  are  being  held: 

(i)  There  is  no  material  lesion  in  the  nervous  system  and  the  disturb- 
ances are  purely  dynamic  or  else  they  are  due  to  some  perturbation  in  the 
cerebral  circulation. 

(2)  There  are  some  material  lesions:  they  may  be  seen  either  macro- 
or  microscopically  in  the  medulla  and  affecting  particularly  the  cardiac 
and  respiratory  centers. 

(3)  To  others  the  reason  of  death  lies  in  anemia  of  the  respiratory 
center.  The  anemia  is  the  result  of  a  compression  of  the  blood  vessels 
produced  by  oedema  of  the  brain.  The  oedema  is  caused  by  a  paralytic 
vaso-dilatation  brought  on  by  the  trauma. 

Concussion  or  shaking,  jarring  of  the  brain  which  will  be  discussed  here 
as  occurring  without  a  gross  injury  of  the  brain  may  result  from  a  direct 
injury,  such  as  a  fall  on  the  head  or  a  blow,  and  from  an  indirect  injury, 
such  as  striking  any  other  part  of  the  body  during  a  sudden  fall,  landing 
suddenly  on  the  feet,  falling  on  the  buttocks,  etc.  In  all  these  accidents 
there  may  be  a  profound  loss  of  consciousness  which  may  develop  imme- 
diately or  else  the  injury  may  be  followed  by  a  very  slight  and  very  brief 
unconsciousness,  also  by  no  loss  of  consciousness.  In  the  latter  cases 
almost  invariably  symptoms  will  develop  within  a  few  hours  or  even  a 
few  days. 

Symptoms. — In  the  severe  cases  after  the  patient  recovers  conscious- 
ness, he  will  present  for  some  time  a  mental  hebetude,  a  vague  expression 
of  the  face.  He  will  complain  of  pain  in  the  head  especially  in  the  occi- 
14  209 


2IO  CONCUSSION   OF    THE  BRAIN 

pital  region,  of  noises  in  the  ears.  He  will  suffer  from  insomnia;  he  is 
irritable  and  at  the  same  time  apathetic.  He  is  mentally  dull,  cannot 
concentrate  his  thoughts.  He  shows  exhaustion  upon  the  least  exertion. 
On  the  least  attempt  to  do  anything,  he  gets  covered  with  perspiration. 
If  he  falls  asleep,  he  awakens  often.  The  pulse  presents  this  characteristic 
feature,  that  it  is  easily  compressible  and  increases  in  rapidity  while  the 
patient  is  under  examination.  Temporary  glycosuria  has  been  observed 
by  some  writers. 

In  the  mild  cases,  without  much  or  any  disturbance  of  consciousness 
the  immediate  effect  of  concussion  will  be  pain  in  the  head,  some  dizziness, 
sometimes  vomiting  and  a  general  sense  of  feeling  weak.  The  pulse  pre- 
sents the  same  peculiarity  as  in  the  severe  cases.  Insomnia,  restlessness, 
photophobia,  loss  of  appetite,  constipation  are  present.  The  mentality 
may  suffer,  but  this  will  be  considered  later. 

Prognosis. — The  outlook  is  variable.  Even  in  the  severest  forms 
recovery  may  follow.  On  the  other  hand  I  have  seen  mild  cases  which 
lasted  months  and  years  and  recovery  remained  incomplete.  In  the 
majority  of  cases,  especially  when  there  is  no  history  of  syphilis  or  alco- 
holism, when  there  are  no  complications,  such  as  fracture  of  the  skull,  also 
when  the  treatment  is  instituted  at  the  earliest  possible  moment,  recovery 
may  be  complete.  An  incomplete  recovery  consists  of  a  continuous  as- 
thenic condition,  loss  of  weight,  pains  in  the  head  and  change  of  disposi- 
tion. Irritability,  dfnculty  of  adapting  oneself  to  surroundings  may  re- 
main for  years. 

In  a  certain  group  of  cases  mental  phenomena  of  a  graver  nature  may 
develop.  Among  the  latter  he  following  disturbances  have  been  observed : 
(i)  Transitory  or  permanent  defects  of  intelligence  especially  of  memory, 
disturbance  of  ability  to  retain  impressions,  retrograde  amnesia.  (2)  De- 
lirium ending  either  in  recovery  or  associated  with  profound  mental  altera- 
tions ending  in  Dementia.  (3)  Hallucinosis.  I  observed  this  manifesta- 
tion in  five  patients.  There  were  no  delusions.  The  hallucinations, 
mostly  visual,  lasted  for  months.  The  patients  finally  recovered. 
(4)  Korsakoff's  psychosis  has  been  observed  in  individuals  free  from 
alcoholism  by  a  number  of  writers.  (5)  Finally  dementia  may  be  the 
ultimate  outcome  of  concussion  of  brain. 

In  children,  when  concussion  occurs  at  an  early  age,  arrest  of  mental 
development  may  occur.  I  have  had  the  opportunity  to  study  several 
children,  whom  I  happened  to  know  before  the  injuries  occurred,  and  a 
decided  change  in  their  mentality  and  intellectual  power  took  place. 
Their  progress  in  mental  development  was  unquestionably  arrested. 

Treatment. — The  most  essential  principle  of  treatment  is  absolute 


CONCUSSION    OF   THE  BRAIN  21 1 

rest  in  bed  which  must  be  instituted  as  early  as  possible  after  the  injury. 
This  should  be  carried  out  not  only  in  the  severe  cases,  but  also  in  the 
cases  of  the  mildest  nature.  The  various  manifestations  should  be  treated 
symptomatically.  Sedatives,  purgatives,  light  diet  and  particularly 
avoidance  of  stimulation,  of  excitement,  of  noise — is  all  that  is  necessary. 
Confinement  to  bed  must  be  as  long  as  possible.  It  is  reiterated  here, 
that  neglect  of  recognizing  the  importance  of  earliest  treatment  may 
render  the  patients  permanent  invalids. 


CHAPTER  XVII 
DISEASES  OF  THE  CEREBELLUM 

The  cerebellum  contains  two  important  portions:  cortex  and  central 
gray  nuclei.  In  the  cortex  we  distinguish:  hemispheres  and  vermis. 
These  two  parts  are  physiologically  independent  of  each  other.  The 
hemispheres  are  in  connection  with  the  cortex  of  the  brain  and  basal 


Fig.  78. — Cerebellar  Tumor   Involving  Left  Hemisphere  and  Vermis.      (Original.) 

ganglia.  The  vermis — with  pons,  medulla  and  spinal  cord.  The  hemi- 
spheres are  concerned  in  voluntary  movements,  the  vermis  is  concerned 
in  coordination  through  the  vestibular  nerve  with  the  nucleus  of  which 
it  is  in  intimate  connection. 


DISEASES    OF   THE   CEREBELLUM  213 

The  function  of  the  cerebellum  is  to  control  the  equilibrium  and  regu- 
late movements.  When  the  cerebellum  is  removed,  motion  and 
equilibrium  are  not  entirely  abolished  because  of  supplementary  action 
of  the  brain  and  of  the  labyrinth. 

Unlike  the  cerebrum,  the  cerebellum  has  its  influence  on  the  muscles 
of  the  same  side  of  the  body. 

Lesions  of  the  cerebellum  may  have  a  slow  and  progressive  course, 
such  as  tumors,  and  an  acute  onset,  such  as  hemorrhage  or  softening. 

Tumors. — Among  the  tumors  of  the  nervous  system  those  of  the 
cerebellum  are  the  most  frequent,  and  as  to  their  nature,  the  tubercular 
form  is  most  common.  All  the  varieties  of  tumors  met  in  the  brain  may 
also  affect  the  cerebellum.  Tubercle  of  the  cerebellum  is  rarely  solitary 
and  never  primary;  it  is  always  accompanied  by  pulmonary  lesions. 

The  point  of  departure  of  cerebellar  tumors  may  be  in  the  peduncles 
or  in  the  meninges  besides  the  cerebellar  tissue  itself.  It  may  be  located 
in  one  of  the  hemispheres  or,  as  it  frequently  happens,  in  the  vermis. 
Cerebellar  tumors  may  be  also  located  in  the  angle  between  the  pons  and 
cerebellum.  They  may  arise  then,  either  in  the  cerebellum  itself  or  in 
the  cranial  nerves.  As  to  the  effect  of  a  tumor  on  the  neighboring  tissue, 
see  the  Pathology  of  Tumors  of  the  Brain. 

Symptoms. — The  general  symptoms  common  to  tumors  of  the  brain 
(see  this  chapter)  are  met  with  here  also.  However  they  present  some 
particularities  deserving  special  mention.  Headache  is  quite  frequently 
occipital  and  very  tenacious;  it  radiates  to  the  neck.  Percussion  over 
the  area  corresponding  to  the  seat  of  the  tumor  provokes  pain.  Vomit- 
ing occurs  quite  early.  Vertigo  is  also  a  precocious  sign  and  persists 
through  the  entire  course  of  the  disease.  It  is  present  even  when  the  pa- 
tient is  at  rest:  sitting  or  lying  down.  Stewart  and  Holmes  observed 
that  when  vertigo  is  present,  objects  and  patient  himself  rotate  from 
the  side  of  the  lesion  to  the  opposite  side. 

Among  the  symptoms  especially  characteristic  of  cerebellar  tumors, 
titubation  occupies  the  first  place.  It  resembles  to  a  great  extent  the 
station  and  gait  of  an  intoxicated  person  and  consists  of  oscillations  of 
the  body  to  the  right  and  left  alternately,  with  a  tendency  to  fall  backward 
or  forward.  The  movements  are  therefore  zigzag-like.  This  condition 
is  observed  in  tumors  of  the  middle  lobe.  When  the  patient  walks,  he 
widely  separates  his  legs.  Standing  is  difficult  because  of  continuous 
oscillations.  Standing  on  the  leg  corresponding  to  the  lesion  is  less 
steady  than  en  the  sound  side.  When  the  patient  shows  a  tendency  to 
walk  toward  one  side  and  holds  the  trunk  and  head  inclined  to  the  same 
side,  the  lesion  is  on  this  side.     This  cerebellar  ataxia  may  affect  not 


214 


DISEASES    OF   THE   CEREBELLUM 


only  the  lower  extremities  (which  is  usually  the  case),  but  also  the  upper. 
When  the  patient  attempts  to  point  to  an  object,  irregular  incoordinate 
movements  appear.  Sometimes  they  are  tremulous,  but  not  like  in 
insular  sclerosis,  because  they  do  not  increase  at  the  end  of  the  act;  they 
rather  disappear. 

Another  disturbance  of  association  of  movements  observed  frequently 
in  cerebellar  diseases  has  been  described  by  Babinski  (1899)  under  the 
name  of  Cerebellar  Asynergy.  When  a  patient  thus  affected  attempts 
to  walk,  the  trunk  does  not  follow  the  legs  in  their  forward  movements 
(Fig.  65).     When  lying  on  his  back  the  patient  attempts  to  sit  up,  he 


Fig.  79. — Cerebellar  Asynergia.     {Bouchard  and  Brissaud.) 

raises  his  legs  and  flexes  the  thighs  on  the  pelvis.  When  seated  on  a 
chair  the  patient  wishes  to  raise  his  leg,  he  flexes  first  the  thigh  on  the 
pelvis  and  then  only  he  elevates  his  leg;  the  latter  movement  is  produced 
very  abruptly;  in  order  to  put  the  foot  back  on  the  ground  the  leg  first 
flexes  over  the  thigh,  then  abruptly  the  latter  becomes  extended  and  the 
foot  reaches  the  floor. 

The  disturbed  associated  movements  just  described  may  be  confined 
to  one  side  and  they  are  then  spoken  of  as  Hemiasynergy.  The  same 
author  also  called  attention  to  the  following  cerebellar  phenomena: 

(1)  Difficulty  of  Regulating  Movements. — When,  for  example,  the 
patient  attempts  to  walk,  he  will  raise  his  foot  higher  than  normally 


DISEASES    OF   THE    CEREBELLUM  21 5 

which  means  excessive  flexion  of  the  thigh  over  the  pelvis.  When  he 
wishes  to  put  his  finger  on  the  nose,  the  former  will  overstep  the  point 
of  destination  and  reach  the  cheek. 

(2)  Adiadochokinesia. — It  consists  of  inability  to  execute  rapid  prona- 
tion and  supination  df  the  hand.  If  it  is  observed  on  one  side  it  usually 
corresponds  to  the  seat  cf  the  lesion. 

(3)  Cerebellar  Catalepsy. — It  consists  of  as  pecial  ability  to  maintain 
a  fixed  position  longer  than  normally.  When  a  patient  lying  on  his  back 
with  his  thighs  flexed  over  the  pelvis,  and  the  legs  over  the  thighs,  raises 
his  limbs,  the  latter  will  at  first  oscillate,  but  in  a  few  moments  the  trunk 
and  the  limbs  will  become  fixed.  This  fixation  may  remain  several  min- 
utes and  is  not  followed  by  fatigue. 

Luciani  called  attention  to  hypotonia  and  asthenia  of  the  limbs  on  the 
side  of  the  lesion  as  characteristic  of  cerebellar  tumors.  General  weakness 
(asthenia)  is  characteristic  of  crebellar  diseases.  It  is  probably  due 
to  inability  to  balance,  to  the  loss  of  equilibrium  of  various  portions  of 
the  body. 

The  position  of  the  head  deserves  special  mention.  In  1908  (/.  Amer. 
Med.  Ass'n)  I  called  attention  to  a  head  phenomenon  which  I  found  of 
localizing  value  in  a  series  of  cases  some  of  which  came  to  autopsy.  It 
consists  of  an  increase  of  vertigo  or  headache  or  of  both  when  the  head  is 
turned  to  the  side  of  the  seat  of  the  cerebellar  lesion. 

The  tendon  reflexes  are  usually  altered.  All  varieties  may  be  present, 
from  diminution  and  loss  to  exaggeration.  A  unilateral  change  does 
not  always  correspond  to  the  seat  of  the  lesion.  Sensations  are  usually 
not  involved. 

Ocular  Symptoms. — They  are  present  in  the  majority  of  cases  and  con- 
sist of  a  more  or  less  pronounced  loss  of  vision.  The  eye-grounds  show 
hyperemia,  oedema,  choked  disc,  optic  atrophy.  The  pupils  are  frequently 
unequal.  Nystagmus  is  almost  a  constant  symptom.  It  is  more  marked 
in  lateral  than  in  vertical  movements  of  the  eyes.  It  is  usually  slow 
and  of  wide  range  toward  the  side  of  the  lesion  but  of  smaller  range  and 
more  rapid  toward  the  normal  side.  Palsies  of  cranial  nerves  are  observed 
in  tumors  of  cerebello-pontine  angle.  Skew  deviation  of  the  eyes  is  some- 
times observed.  It  consists  of  one  eye  looking  downward  and  inward, 
the  other  outward  and  upward. 

Articulation  of  speech  is  not  infrequently  affected  in  cerebellar 
tumors.  It  is  slow  and  explosive,  but  not  quite  as  explosive  as  in 
insular  sclerosis. 

Cerebellar  tumor  frequently  causes  an  increase  of  cerebro-spinal 
fluid  (hydrocephalus) .     This  is  probably  the  cause  of  the  mental  disturb- 


2l6  DISEASES    OP   THE   CEREBELLUM 

ances,  apathy  and  hebetude  observed  during  the  last  period  of  the 
disease. 

The  symptoms  of  compression  of  the  neighboring  tissue  are  various. 
Hemiplegia,  hemiparesis  or  crossed  hemiplegia  may  be  observed.  The 
hemiplegia  is  not  spastic,  the  face  is  not  involved  and  hypotonia  of  the 
muscles  is  present  on  the  affected  side.  The  toe  phenomenon  and 
ankle-clonus  may  be  absent.  The  cranial  nerves,  particularly  the  third 
and  sixth,  may  become  involved. 

Course.  Duration.  Prognosis. — Headache  and  vertigo  are  usually 
the  first  symptoms.  They  may  persist  for  several  months  before  the 
titubation  makes  its  appearance.  Rarely  amaurosis  is  wanting.  Asthenia 
also  appears  early.  Gradually  convulsions  develop.  The  duration  of  the 
disease  is  indefinite.  It  may  last  from  several  months  to  several  years, 
although  usually  it  runs  a  rapid  course.  The  termination  is  fatal  in  cases 
which  are  not  operated  upon,  although  a  few  cases  of  recovery  of  tubercu- 
loma of  the  cerebellum  have  been  reported.  Rapid  termination  occurs 
in  cases  of  compression  of  the  medulla. 

Diagnosis. — In  a  certain  number  of  cases  the  diagnosis  cf  a  cerebellar 
tumor  presents  great  difficulties.  This  is  particularly  seen  in  the  initial 
stage,  when  only  general  symptoms  are  present,  as  it  is  well  known  the 
latter  are  common  also  to  cerebral  growths.  When  iu  addition  to  the  lat- 
ter bulbar  symptoms  appear,  the  presumption  is  in  favor  of  a  cerebellar 
involvement.  In  cases  in  which  in  addition  to  the  general  signs  even  one 
of  the  special  symptoms  should  make  its  appearance,  the  diagnosis  will 
readily  be  made.  When  the  cerebellar  involvement  is  decided  upon,  the 
question  of  its  exact  seat  must  be  solved,  as  operative  procedures  depend 
upon  it.  When  the  tumor  is  unilateral,  the  characteristic  symptoms  will 
be  present  on  the  same  side.  A  tumor  of  the  median  lobe  will  give  bi- 
lateral symptoms.  Particular  stress  must  be  laid  upon  hemiasynergy, 
upon  the  unilateral  occipital  headache  with  tenderness  on  percussion  of 
the  occiput,  upon  the  position  of  the  head,  upon  nystagmus  and  other 
ocular  symptoms,  upon  the  state  of  the  reflexes  on  one  or  the  other  side, 
upon  the  state  of  the  reflexes  on  one  or  the  other  side,  upon  involvement  of 
one  or  several  cranial  nerves  on  one  side.  All  these  informations  are  of 
great  utility,  as  the  symptoms  usually  correspond  to  the  seat  of  the  tumor. 

As  to  other  diseases  with  which  a  cerebellar  affection  may  be  con- 
founded, tabes  particularly  must  be  mentioned.  Ataxia  and  loss  of  reflexes 
may  be  present  in  both,  but  the  zigzag  movements  of  a  cerebellar  is  not 
present  in  a  tabetic;  the  raising  of  the  feet  high  and  dropping  them  with 
force  on  the  ground  are  characteristic  only  of  the  tabetic.  Moreover 
other  symptoms  will  help  to  decide  the  diagnosis. 


DISEASES    OP    THE    CEREBELLUM  217 

In  Meniere's  disease  there  are:  vomiting,  vertigo  and  even  titubation, 
but  absence  of  headache  and  of  ocular  symptoms  will  render  the  diagnosis 
comparatively  easy. 

Treatment. — In  cases  in  which  syphilis  is  suspected,  mercurials  and 
iodides  should  be  tried.  Relief  and  removal  of  the  distressing  symptoms 
for  a  long  time  may  follow  from  specific  treatment  in  cases  of  cerebellar 
gummata.  In  two  of  my  cases  the  ataxia,  vomiting  and  headache  disap- 
peared completely  under  iodides.  Even  in  tubercular  tumors  of  the  cere- 
bellum iodides  will  be  of  great  value,  as  I  could  ascertain  it  in  one  of  my 
cases,  in  which  all  the  symptoms  except  the  blindness- disappeared.  In 
the  largest  majority  of  cases  surgical  treatment  alone  is  applicable. 

In  the  chapter  on  Tumors  of  the  Brain  operative  procedures  are 
fully  discussed  with  especial  reference  to  decompression ;  the  latter  will 
at  least  decrease  the  intracranial  pressure  and  thus  relieve  pain,  making 
therefore  life  more  tolerable.  But  where  particularly  lies  the  benefit  of 
operation  is  the  prevention  of  blindness  and  restoration  of  sight.  In 
one  of  my  recent  cases  the  patient  was  relieved  from  his  headache,  choked 
disc  disappeared,  sight  was  restored  and  this  condition  lasted  almost 
two  years  when  he  died  from  pneumonia.  A  large  part  of  the  skull  of  the 
occipital  region  should  be  removed.  In  dcubtful  cases  as  to  the  localiza- 
tion, it  has  been  advised  to  trephine  first  the  right  temporal  region,  thus 
relieving  general  intracranial  pressure.  If  the  cerebellar  symptoms  remain 
unaltered,  another  operation  over  the  occipital  bone  should  be  attempted 
ater  (sub tentorial  operation). 

TUMORS  OF  PONTO -CEREBELLAR  ANGLE 

These  tumors  originate  either  in  the  meninges  or  more  frequently 
in  the  cranial  nerves  of  the  base  of  the  brain,  particularly  in  the  eighth 
nerve,  but  sometimes  also  in  the  fifth,  seventh,  ninth,  tenth,  and  eleventh 
nerves.  They  are  usually  solid,  resistant  and  small.  They  contain 
fibrous  elements  (fibroma,  fibro-sarcoma,  neuro-fibroma) .  In  one  of 
my  cases  there  was  a  cyst  {Old  Dom.  J.  of  Med.  and  Surgery,  1910). 
They  are  benign  tumors  and  their  extirpation  if  successful,  usually  leads 
to  recovery.  They  occupy  the  space  between  the  lateral  border  of  the 
pons  and  the  cerebellar  hemisphere.  This  space  corresponds  to  the  pos- 
terior surface  of  the  petrous  bone  at  the  level  of  the  auditory  meatus, 
into  which  enter  the  seventh  and  eighth  nerves. 

Symptoms. — General  symptoms  of  intracranial  pressure  are  present, 
viz.  Headache,  vomiting,  stasis  of  papillae.  The  latter  is  an  early  dis- 
turbance.    It  is  frequently  followed  by   optic   atrophy.     Headache  is 


2l8  DISEASES    OF   THE   CEREBELLUM 

frequently  localized  in  the  occipital  region.  Pressure  of  the  cerebellum 
produces  the  characteristic  cerebellar  symptom  group  (see  above). 
Vertigo  deserves  special  mention.  Steward  and  Holmes  assert  that 
objects  seem  to  move  from  the  side  of  the  lesion  to  the  normal  side,  but 
the  subjective  sense  of  rotation  of  the  patient  himself  is  in  the  opposite 
direction.  In  tumors  of  the  cerebellum  the  reverse  condition  of  the  latter 
is  observed.  Symptoms  referable  to  compression  of  the  nerve  trunks 
at  the  base  are  the  most  characteristic  feature.  The  auditory  nerve 
particularly  suffers  and  this  is  manifested  by  noises  in  the  ear  and  soon 
by  deafness  on  the  affected  side,  while  the  ear  itself  is  intact.  Next  in 
frequency  are  the  facial  and  trigeminal  nerves.  The  involvement  of  the 
former  is  manifested  by  a  hemispasm  and  more  frequently  by  paresis  of 
the  face.  Hyperesthesia,  neuralgia  or  else  anaesthesia,  also  corneal  anaes- 
thesia with  loss  of  the  corneal  reflex  are  signs  of  involvement  of  the  fifth 
nerve.  More  rarely  the  sixth,  ninth,  and  tenth  nerves  are  affected. 
Corneal  areflexia,  hypoacousia  and  paresis  of  associated  movements 
of  the  eyes  are  according  to  Oppenheim  characteristic  of  ponto-cerebellar 
tumors. 

Operation  is  the  only  treatment.  Among  various  methods  that 
of  Krause  is  the  most  accepted.  He  operates  on  the  occipital  region 
and  exposes  the  cerebellar  hemispheres.  The  vicinity  of  the  cerebellum 
and*  of  the  medulla  renders  the  operation  very  serious.  The  mortality 
indeed  is  very  great.  In  so-called  successful  cases,  the  most  distressing 
symptoms  disappear,  but  the  auditory  manifestations  especially  are  very 
little,  if  any,  influenced  by  the  operation.  Vision  may  be  only  improved, 
except  when  there  is  already  optic  atrophy.  In  spite  of  the  gravity 
operations  are  justifiable.  Some  very  successful  cases  have  been  reported. 
Recurrences  of  the  tumors  are  not  known. 

ABSCESS  OF  THE  CEREBELLUM 

Etiology.— Apart  from  metastatic  abscesses,  the  most  frequent  cause 
is  suppuration  of  the  temporal  bone.  The  latter  is  a  common  occurrence 
in  chronic  otitis  media.  Occasionally  an  acute  otitis  media  may  be  the 
cause  of  a  cerebellar  abscess.  The  otitis  itself  may  develop  in  the 
course  of  grippe,  of  pharnygitis,  of  exanthematous  infectious  diseases. 
Speaking  generally,  any  cause  leading  to  caries  of  the  temporal  bone, 
and  its  petrous  portion  particularly,  will  produce  a  cerebellar  abscess. 

Pathology.— When  the  abscess  is  seated  within  the  cerebellar  sub- 
stance, the  surrounding  nervous  tissue  may  be  reduced  to  a  minimum. 
As  the  abscess  is  usually  secondary  to  caries  of  the  neighboring  bone 
(particularly  temporal),  the  meninges  will  be  found  diseased.     Phlebitis 


DISEASES    OF   THE   CEREBELLUM  2IQ 

of  the  sinuses,  especially  of  the  lateral,  is  a  frequent  finding  and  throm- 
bosis is  therefore  equally  frequent.  The  surrounding  cerebellar  tissue 
undergoes  softening  and  destruction.  The  pus  is  thick,  greenish  and 
fetid.  It  contains  the  usual  microbes  of  suppuration,  viz.  streptococcus, 
staphylococcus  or  pneumococcus. 

Symptoms. — In  acute  stage  the  otorrhea  and  the  deafness,  which 
have  been  present  for  a  more  or  less  long  period,  become  suddenly  com- 
plicated by  grave  cerebral  symptoms:  coma  with  general  symptoms  of 
infection.     The  characteristic  cerebellar  symptoms  cannot  be  revealed. 

In  the  chronic  stage  the  special  cerebellar  symptoms,  described  in 
"Tumors  of  the  Cerebellum,"  are  easily  observed.  In  order  to  avoid 
repetition  the  reader  is  referred  to  that  chapter.  A  few  special  manifes- 
tations are  worth  mentioning.  Rigidity  of  the  neck  and  retraction  of 
the  head,  slow  nystagmus,  pronounced  vertigo  upon  the  least  movement 
of  the  head,  frequent  occurrence  of  optic  neuritis — are  all  not  infrequently 
observed  in  abscess  of  the  lateral  lobe  of  the  cerebellum. 

An  abscess  may  compress  the  neighboring  portions  of  the  nervous 
system.  A  crossed  paralysis,  viz.  involvement  of  any  of  the  cranial  nerves 
on  one  side  and  of  the  extremities  on  the  opposite  side,  is  indicative  of 
pressure  at  the  base  of  the  brain. 

An  abscess  may  remain  silent  for  a  long  time,  when  it  is  not  seated 
in  the  middle  lobe  and  strictly  limited.  Most  of  the  time  it  spreads  to 
the  neighboring  tissue  and  produces  great  damage  in  the  nervous  tissue. 

Prognosis. — It  is  identical  with  that  of  cerebellar  tumors  in  the 
chronic  stage,  but  it  is  very  serious  in  the  acute  stage. 

Diagnosis. — In  the  chronic  stage  it  will  be  extremely  difficult  to 
differentiate  the  disease  from  tumor.  Otorrhea  or  a  diseased  condition 
of  the  mastoid  are  suggestive  rather  of  abscess  than  of  tumor.  In  the 
acute  stage  the  diagnosis  presents  sometimes  insurmountable  difficulties, 
as  apart  from  the  ear  symptoms  which  may  be  overlooked,  there  is  very 
little  to  make  a  diagnosis.     The  general  condition  is  that  of  meningitis. 

Treatment. — Operative  intervention  is  the  only  treatment  and  it 
should  be  done  as  early  as  possible.  A  large  opening  should  be  made 
in  the  skull  and  the  pus  evacuated,  if  the  abscess  is  on  the  surface  of  the 
cerebellum;  a  puncture  should  be  made  in  the  cerebellum,  if  the  abscess 
is  within  the  latter. 

CEREBELLAR  HEREDO-ATAXIA 

u  Under  this  name  P.  Marie  described  a  form  of  cerebellar  incoordina- 
tion hereditary  in  character  and  occurring  in  several  members  of  the  same 
family. 


2  20  DISEASES    OF   THE    CEREBELLUM 

Pathology. — The  characteristic  condition  of  this  affection  is  atrophy 
of  the  cerebellum,  which  is  due  to  arrested  development.  The  autopsy 
records  show  that  in  some  cases  in  addition  to  the  cerebellar  atrophy  there 
was  also  atrophy  of  the  medulla  and  of  the  spinal  cord.  In  Marie's  first 
case  there  was  also  sclerosis  of  Goll's,  Gower's  and  direct  cerebellar  tracts, 
finally  marked  diminution  of  the  middle  cerebellar  peduncles.  In  some 
cases  the  changes  were  found  to  affect  only  the  gray  matter  of  the  cere- 
bellum.    The  cerebellum  is  rarely  alone  affected. 

Symptoms. — The  striking  feature  is  the  disturbance  of  coordination. 
The  lower  extremities  are  affected  long  before  the  upper.  The  usual 
cerebellar  titubation  with  asynergia,  asthenia,  scoliosis,  ocular  disorders, 
exaggerated  reflexes  and  integrity  of  sensory  functions — otherwise  speak- 
ing, all  the  symptoms  found  in  cerebellar  diseases  (see  above)  are  also  found 
in  Marie's  disease. 

The  characteristic  features  lie  in  the  evolution  of  the  symptoms.  The 
onset  is  gradual.  In  some  cases  neurasthenic  symptoms  precede  the  disturb- 
ance of  equilibrium.  The  patient  complains  of  headache,  pain  in  the  back 
and  general  fatigue.  The  first  signs  appear  early  in  life,  generally  between 
fifteen  and  twenty-five  years  of  age.  Disturbance  of  equilibrium,  which  is 
soon  followed  by  a  typical  cerebellar  ataxia,  are  the  initial  symptoms.  They 
are  soon  followed  by  a  disturbance  of  speech  and  voice :  the  speech  is 
irregular,  each  word  is  accentuated  and  precipitated;  the  voice  is  monoto- 
nous and  guttural.  When  the  patient  speaks,  there  is  noticeable  an 
exaggerated  contraction  of  the  muscles  of  the  face.  In  the  course  of  the 
malady  incoordination  of  the  upper  extremities  usually  develops.  Their 
movements  become  uncertain;  a  fine  intention  tremor  is  quite  frequently 
present,  so  that  delicate  acts,  such  as  writing,  threading  a  needle,  etc.,  are 
almost  impossible. 

The  psychic  faculties  are  usually  altered.  Impairment  of  intelli- 
gence, of  memory,  irritability  and  indifference  are  the  main  disturbances 
observed. 

The  disease  is  invariably  progressive,  although  it  may  remain  station- 
ary for  a  long  time.  In  its  lastp  eriod  there  is  absolute  physical  impotence : 
the  patient  is  confined  to  bed  and  usually  dies  from  some  intercurrent 
disease. 

Diagnosis. — The  history,  the  onset,  the  gradual  development  of  the 
symptoms,  the  family  character  of  the  disease  will  enable  one  to  differen- 
tiate it  from  tumors,  hemorrhages  or  abscesses  of  the  cerebellum;  also 
from  cerebral  diplegia.  Some  difficulty  is  found  sometimes  in  distinguish- 
ing it  from  Friedreich's  ataxia.  In  favor  of  heredo-cerebellar  ataxia  will 
be  the  family  character,  the  age  at  which  it  occurs,  the  slow  development, 


DISEASES    OF   THE    CEREBELLUM  221 

cerebellar  gait,  asynergia,  incoordination  of  the  upper  extremities,  ataxia 
in  writing,  nystagmus,  the  peculiar  facial  mimicry,  intention  tremor, 
finally  increased  knee-jerks  with  ankle-clonus.  The  condition  of  the  knee- 
jerks  is  particularly  important,  as  their  abolition  is  characteristic  of  Fried- 
reich's ataxia.  The  latter  affection  may  present  sometimes,  especially  at 
the  beginning,  normal  reflexes;  on  the  other  hand,  in  Marie's  disease,  when 
the  lesion  reaches  the  cord,  the  reflexes  may  be  abolished.  In  such  cases 
the  other  symptoms  will  aid  in  making  a  diagnosis. 

Etiology. — A  neurotic  family  history  is  not  infrequently  obtained. 
Alcoholism,  tuberculosis  in  parents  are  reported  by  some  observers.  Con- 
sanguinity and  syphilis  have  been  noted  in  a  limited  number  of  cases. 
Some  writers  mention  also  infectious  diseases  and  traumatism.  The  fe- 
male sex  is  apparently  more  frequently  affected  than  the  male.  Members 
of  the  same  family  become  frequently  affected  at  the  same  age. 

Treatment. — Antisyphilitic  treatment  may  be  tried,  but  in  view  of  the 
character  of  the  lesion  (see  Pathology)  no  results  can  be  expected  from 
medications. 

HEMORRHAGE  AND  SOFTENING  OF  THE  CEREBELLUM 

Hemorrhages  are  quite  rare.  In  ioo  cases  of  cerebellar  diseases  de- 
scribed by  Krauss  there  was  one  case  of  hemorrhage.  In  187  cases  of 
apoplexy  Starr  found  only  four  cases  in  the  cerebellum.  In  the  majority 
of  cases  they  have  been  found  in  the  hemispheres.  Minute  hemorrhages 
are  less  frequently  observed  than  large  ones.  When  a  hemorrhage  occurs, 
the  blood  is  apt  easily  to  break  through  the  cerebellum  and  invade  the 
fourth  ventricle.  The  superior  and  inferior  cerebellar  peduncles  are  rarely 
the  seat  of  hemorrhages,  but  the  middle  peduncle  may  be  invaded  by  a 
hemorrhage  originating  in  the  pons. 

Softening  of  the  cerebellum  is  extremely  rare  for  the  following  ana- 
tomical reasons:  (1)  the  angle  formed  by  the  basilar  and  cerebellar  arte- 
ries interferes  with  the  formation  of  embolism  in  the  latter;  (2)  thrombosis 
is  very  rare  in  the  cerebellum. 

Symptoms  (of  Hemorrhage). — The  prodromal  symptoms  and  the  onset 
itself  are  identical  to  those  of  hemorrhage  in  the  cerebrum.  Pain  in  the  oc- 
cipital region,  persistent  vomiting  are  very  early  symptoms.  If  the  patient 
does  not  die  immediately  after  the  loss  of  consciousness,  he  remains  coma- 
tose. When  consciousness  is  regained,  the  striking  differential  sign  will  be 
the  absence  of  hemiplegia,  which  on  the  contrary  is  so  constant  in  apoplexy 
of  cerebral  origin.  However,  Mann  (Monatschr.  fur  Psychiatrie  u.  Neurol- 
ogie,  1902)  described  cerebellar  hemiplegia  on  the  side  of  the  lesion  which 


22  2  DISEASES    OF   THE   CEREBELLUM 

presents  the  following  characteristics:  it  affects  all  the  muscles  of  the 
both  limbs,  while  in  cerebral  hemiplegia  only  certain  muscular  groups 
are  affected,  such  as  flexors  of  the  leg  and  of  the  foot,  extensors  of  the  hand; 
moreover,  there  is  no  contracture;  the  reflexes  are  exaggerated;  there  is 
no  rigidity,  no  ankle-clonus,  no  toe  phenomenon. 

After  the  patient  recovered  from  the  immediate  effects  of  the  attack, 
there  is  noticed  an  extreme  general  weakness  (not  a  paralysis),  so  that  the 
patient  cannot  even  remain  seated.  Soon  the  other  symptoms  of  cere- 
bellar diseases  make  their  appearance,  viz.  cerebellar  ataxia,  etc.  (see  Cere- 
bellar Tumors) .  If  paralysis  develops,  it  is  almost  never  at  the  beginning, 
but  later,  and  it  is  due  to  pressure  upon  the  pons  and  medulla. 

In  softening  the  onset  will  not  be  sudden,  but  progressive.  Later 
the  symptoms  will  be  identical  with  those  of  hemorrhage. 

In  view  of  a  considerable  anastomosis  in  the  cerebellar  arterial  supply, 
softening  is  usually  confined  to  a  small  area. 


CHAPTER   XVIII 

DISEASES  OF  THE  MEDULLA,  PONS  AND  THE  FOURTH 

VENTRICLE 

These  three  portions  of  the  nervous  system  contain  extremely  impor- 
tant elements.  The  sensory  and  motor  pathways,  the  nuclei  of  ten  of  the 
cranial  nerves,  centers  for  some  of  the  vegetative  functions — are  all  located 
here.  In  diseases  therefore  of  this  area  the  symptomatology  is  complex. 
There  are,  however,  a  few  groups  of  symptoms  which  constitute  special 
forms  of  diseases  characteristic  of  involvement  of  this  area  of  the  nervous 
system. 

A.  ACUTE  SUPERIOR  POLIOENCEPHALITIS   (HEMORRHAGIC) 

Pathology. — The  lesion  consists  of  an  inflammation  with  hemorrhages 
in  the  gray  matter  of  the  aqueduct  of  Sylvius.  The  latter  is  therefore 
found  in  a  state  of  softening.  Microscopically  are  seen  dilated  blood  ves- 
sels, the  perivascular  spaces  are  filled  with  blood,  a  leucocytic  infiltration 
is  marked.  Sometimes  this  pathological  process  extends  forward  into  the 
floor  of  the  third  ventricle  and  backward  into  the  fourth  ventricle.  The 
nucleus  of  the  third  nerve,  also  the  fibers  emerging  from  it,  are  therefore 
the  main  seat  of  the  lesion. 

Etiology. — The  most  frequent  cause  is  chronic  alcoholism.  Other 
intoxications,  as  carbonic  acid,  sulphuric  acid,  infectious  diseases,  alimen- 
tary intoxications  (fish,  meat,  etc.) .  are  sometimes  followed  by  superior 
polioencephalitis. 

Symptoms.- — The  onset  is  acute.  In  the  course  of  chronic  alcoholism 
or  an  infectious  disease  the  patient  is  suddenly  taken  with  headache  and 
vertigo.  Soon  somnolence  and  delirium  make  their  appearance.  Rapidly 
palsies  of  the  eye  muscles  develop. 

Most  cf  the  time  both  eyes  are  involved,  but  the  degree  of  palsy  is 
unequal.  Sometimes  an  associated  paralysis  is  observed;  either  both 
external  recti  or  both  internal  recti  are  affected.  Ptosis  is  not  frequent. 
Wernicke  (whose  name  is  attached  to  the  disease)  claims  that  the  sphincter 
of  the  iris  is  never  involved.  I  have  records  of  two  personal  cases  with 
palsy  of  this  sphincter. 

Optic  neuritis,  nystagmus  may  occur. 

223 


2  24  DISEASES    OF   MEDULLA,    PONS   AND    FOURTH  VENTRICLE 

In  addition  to  the  ocular  disturbances,  which  are  the  characteristic 
feature  of  the  disease,  other  symptoms  are  not  infrequently  observed. 
Ataxia,  unilateral  paralysis  of  the  extremities,  disturbance  of  speech, 
exaggeration  or  abolition  of  the  tendon  reflexes,  disturbances  of  degluti- 
tion and  of  mastication  may  be  met  with. 

As  the  inflammation  may  become  diffuse  and  be  ascending  or  de- 
scending or  both,  cerebral  as  well  as  spinal  symptoms  may  be  associated. 
Polioencephalitis,  superior  and  inferior  (see  further),  and  poliomyelitis 
are  not  infrequently  combined  (polioencephalomyelitis).  The  exten- 
sion of  the  inflammatory  process  from  the  gray  to  the  white  matter  of 
the  pons  and  medulla  (which  is  not  rare)  will  explain  the  above-mentioned 
additional  clinical  phenomena. 

Course,  Duration  and  Prognosis.— The  evolution  of  the  symptoms 
is  rapid.  In  the  majority  of  cases  the  duration  is  from  eight  to  fourteen 
days,  although  in  one  case  it  lasted  sixteen  weeks.  Recovery  was  re- 
poited  in  a  few  cases.  In  one  of  my  cases  the  palsy  of  the  third  and 
fourth  nerves  has  been  in  existence  two  and  one-half  years.  The  patient  is 
still  living.  The  onset  in  this  particular  case  was  apoplectiform  in  nature 
with  loss  of  consciousness.  The  palsy  affected  all  the  muscles  supplied 
by  the  third  and  fourth  nerves.  In  another  case,  a  child  of  ten  months, 
there  was  also  loss  of  consciousness  with  involvement  of  the  third,  fourth 
and  sixth  nerves  simultaneously.  The  child  is  now  three  years  old, 
enjoying  good  health  and  the  nerve  palsy  has  greatly  improved.  In 
both  cases  the  paralysis  is  bilateral,  but  unequally  distributed  to  vari- 
ous muscles  of  the  eye  globes.  In  the  case  of  the  child  the  condition 
was  preceded  by  an  attack  cf  cough  for  a  period  of  four  or  five  days, 
evidently  of  infectious  nature.  In  the  first  case  the  woman  was  ad- 
dicted to  the  use  of  alcohol.  She  was  thirty-five  years  of  age.  Both 
patients  present  a  nystagmus  on  lateral  movements.  The  tendon  reflexes 
were  exaggerated  in  the  woman. 

Treatment. — The  general  symptoms  may  be  relieved  by  the  usual 
medications,  as  for  example  the  headache  by  opium,  morphia  or  coal-tar 
products.  Bleeding,  purgatives  and  diaphoretics  may  be  tried.  Little 
reliance  should  be  placed  upon  drugs.     Iodides  may  do  some  good. 

B.  CHRONIC   SUPERIOR  POLIOENCEPHALITIS  (PROGRESSIVE  NUCLEAR 

OPHTHALMOPLEGIA) 

The  disease  is  characterized  by  a  slow  but  progressive  paralysis  of 
the  muscles  of  the  eye. 

Pathology. — Atrophy  of  the  motor  nuclei  cf  the  eye  is  the  character- 


DISEASES    OF   MEDULLA,    PONS    AND    FOURTH  VENTRICLE  225 

istic  lesion.  Chromatolysis  with  formation  of  vacuoles,  also  marked 
pigmentation  of  the  cells  of  the  nuclei,  are  the  first  stage  of  the  affection. 
Later  on  the  cells  disappear  and  the  nuclei  are  in  a  state  of  atrophy. 

In  some  cases  the  nuclei  of  all  the  motor  nerves  of  the  eye  are  affected, 
in  others  only  the  nucleus  of  the  third  nerve  is  diseased.  Secondarily 
the  roots  emanating  from  the  nuclei,  the  nerves  themselves  and  the 
muscles  innervated  by  the  latter  undergo  degeneration. 

Etiology. — Infections,  intoxications,  syphilis  may  be  the  causes  of  the 
malady.  It  is  occasionally  observed  in  the  course  of  organic  diseases 
of  the  nervous  system :  disseminated  sclerosis,  tabes,  paresis. 

Symptoms. — The  onset  is  insidious.  While  there  is  no  constancy 
in  the  order  of  involvement  of  individual  ocular  muscles,  nevertheless 
ptosis  and  diplopia  are  the  first  symptoms  in  the  majority  of  cases.  As 
at  the  beginning,  the  palsy  is  not  complete,  the  movements  of  the  eye 
globe  in  certain  directions  are  yet  possible,  but  only  after  an  effort.  The 
facics  of  the  patient  is  quite  characteristic.  As  the  upper  eyelids  are 
lowered,  he  holds  his  head  thrown  backward  in  order  to  be  able  to  see 
objects;  the  forehead  is  wrinkled  as  the  frontal  muscles  are  trying  to 
raise  the  palsied  eyelids.  The  movements  of  the  eye  depend  upon  the 
muscles  involved.  In  an  advanced  stage  of  the  disease  the  eyes  become 
immobile,  and  in  order  to  see  on  the  right  or  on  the  left,  the  head  must 
be  turned.  External  ophthalmoplegia  is  frequently  associated  with 
internal  ophthalmoplegia.  In  such  cases  the  pupil  ceases  to  react  to 
light  or  accommodation. 

Course.  Duration.  Prognosis. — When  the  lesion  remains  ccnfined 
to  the  ocular  nuclei,  the  disease  may  last  an  indefinite  number  of  years. 
The  disease  may  begin  unilaterally  and  remain  confined  to  the  same  side. 
When  it  has  a  descending  course  and  becomes  complicated  by  an  infe- 
rior polioencephalitis  (see  further,  death  may  ensue  in  a  very  short 
time.  At  all  events  a  nuclear  ophthalmoplegia  is  an  incurable  and  grave 
affection. 

Diagnosis. — From  the  acute  form  it  will  be  distinguished  by  the  ab- 
sence of  general  symptoms  (see  above),  by  the  slow  course.  Opthalmo- 
plegia  may  be  due  to  a  lesion  at  the  base  of  the  brain,  but  in  the  latter 
case  other  cranial  nerves  are  usually  involved.  In  the  course  of  infectious 
diseases  or  intoxications  external  ophthalmoplegia  may  be  observed,  but 
usually  there  are  also  palsies  of  the  limbs,  of  pharynx,  larynx,  of  face. 
These  are  cases  of  multiple  neuritis  and  bear  usually  a  favorable  progno- 
sis :  improvement  is  almost  invariable. 

In  Myasthenia  gravis  (see  this  chapter)  there  is  also  a  weakness  of  the 
masticator  muscles  and  the  general  asthenia  is  very  striking. 
is 


226  DISEASES    OF   MEDULLA,   PONS    AND    FOURTH  VENTRICLE 

Paralysis  of  the  muscles  of  the  eye  may  be  the  result  of  a  disease  of  the 
orbit.     In  such  cases  the  diagnosis  is  easy. 

Treatment. — There  is  practically  no  medication  to  rely  upon.  Mer- 
cury and  iodides  should  be  tried  even  in  cases  without  a  history  of  syphilis. 
Electricity  may  also  be  applied. 

C.  ACUTE  INFERIOR  POLIOENCEPHALITIS   (ACUTE  BULBAR  PALSY) 

Pathology. — The  lesion  is  identical  to  that  of  acute  superior  polioen- 
cephalitis (see  above).  The  difference  lies  only  in  the  seat.  Hemor- 
rhages and  softening  are  the  immediate  causes.  Here  all  other  nuclei,  ex- 
cept those  of  the  third,  fourth  and  sixth  cranial  nerves,  are  involved  of 
only  some  of  them.  A  not  infrequent  occurrence  is  the  association  of 
both  forms  of  polioencephalitis. 

Etiology. — Infectious  diseases  and  alimentary  intoxications  are  reported 
to  be  the  causes  of  the  disease,  which  is,  however,  quite  rare. 

Symptoms. — The  onset  is  sudden  or  rapid  and  characterized  by  general 
symptoms,  such  as  headache,  chills,  fever  and  pain  in  the  neck.  Loss  of 
consciousness  is  usually  present  in  hemorrhages,  but  not  in  softening. 
Soon  the  patient  develops  difficulty  in  swallowing  and  articulation;  other- 
wise speaking,  the  picture  is  that  of  labio-glosso-laryngeal  paralysis  (see 
next  chapter).  The  tongue,  lips,  palate  are  paralyzed.  The  food  is 
regurgitated  through  the  nostrils  and  the  patient  is  threatened  with  suffo- 
cation. The  pulse  and  respiration  are  irregular.  Trismus  is  according 
to  Joffroy  a  characteristic  symptom.  A  comatose  state  sets  in  rapidly 
and  death  is  the  usual  termination. 

Termination.  Prognosis. — The  disease  usually  lasts  from  two  to  six 
days.     Some  exceptional  cases  of  recovery  have  been  reported. 

Treatment. — Revulsion  and  counterirritation  on  the  neck,  also  purga- 
tion, are  the  only  means  for  the  disease,  which  is  almost  invariably 
fatal. 


D.  CHRONIC  INFERIOR  POLIOENCEPHALITIS  (CHRONIC  BULBAR  PALSY) 
(LABIO-GLOSSO-LARYNGEAL  PARALYSIS) 

The  disease  is  characterized  by  a  paralysis  of  the  muscles  of  the  lips, 
tongue,  pharnyx  and  larynx. 

Pathology. — The  lesion  consists  of  a  primary  and  progressive  degenera- 
tion of  the  nuclei  of  origin  of  the  cranial  nerves,  situated  in  the  lower  half 
of  the  medulla,  viz.  those  of  the  seventh,  ninth,  tenth,  eleventh  and  twelfth 
pairs.     The  alterations  are  most  marked  in  the  nucleus  of  the  hypoglossus 


DISEASES    OF   MEDULLA,   PONS   AND   FOURTH  VENTRICLE  227 

(twelfth).  They  consist  of  diminution  or  disappearance  of  the  chromato- 
philic  substance  of  the  cells,  of  appearance  of  pigment  within  the  cells,  of  a 
displacement  or  disappearance  of  the  nucleus  or  nucleolus,  finally  of  atrophy 
of  all  the  prolongations  of  the  cells.  The  nerve-roots  emanating  from  the 
nuclei  therefore  appear  very  thin.  The  degree  of  involvement  is  not 
equal  in  all  the  cells  of  the  same  nucleus  and  in  various  nuclei. 

As  to  the  participation  of  the  white  matter  of  the  medulla  in  the 
pathologic  process,  all  the  writers  are  not  agreed.  Some  claim  (Ray- 
mond, Leyden,  Dejerine)  that  the  labio-glosso-laryngeal  paralysis  of 
Duchenne  is  not  an  autonomous  disease,  but  is  almost  always  followed 
by  an  involvement  of  the  pyramidal  bundles  to  constitute  amyotrophic 
lateral  sclerosis.  In  the  ascending  course  of  the  latter  disease  bulbar 
symptoms  almost  invariably  appear,  but  there  is  also  a  form  in  which 
the  disease  begins  with  labio-glosso-laryngeal  palsy  (see  Amyotrophic 
Lateral  Sclerosis). 

The  muscles  in  which  the  affected  cranial  nerves  are  distributed  also 
undergo  atrophy,  viz.  those  of  the  tongue,  lips,  pharynx  and  larynx. 

Etiology. — Syphilis,  fatigue,  Bright's  disease  have  been  reported  in 
the  histories  of  some  patients,  but  there  are  no  positive  data  as  to  the  true 
etiological  factors  of  this  affection. 

Bulbar  paralysis  may  occur  in  the  course  of  amyotrophic  lateral 
sclerosis,  tabes,  multiple  sclerosis  and  syringomyelia. 

Symptoms. — The  onset  is  insidious  and  slow.  There  is  usually  a  brief 
prodromal  stage  during  which  the  patient  complains  of  pain  in  the  neck 
and  a  numbness  of  the  pharynx.  Gradually  the  lips,  larynx  and  the  tongue 
become  paralyzed.  The  latter  particularly  is  affected  first  in  the  major- 
ity of  cases.  At  the  beginning  there  is  only  a  weakness  in  the  movements 
of  the  tongue,  but  it  keeps  on  increasing  until  complete  immobility  is 
established.  The  letters  that  require  the  cooperation  of  the  tongue  aer 
imperfectly  pronounced.  The  speech  is  therefore  impaired  (dysarthria). 
The  tongue  is  flat,  dixninished  in  size  and  presents  fine  fibrillary  contrac- 
tions, also  reactions  of  degeneration.  In  an  advanced  stage  the  atrophy  cf 
the  tongue  is  very  marked;  it  then  presents  a  depression  and  on  palpation 
it  is  very  soft.  The  speech  is  impossible  (anarthria).  Mastication  and 
deglutition  are  difficult. 

The  lips  follow  the  tongue.  It  is  the  orbicularis  muscle  that  is  first 
affected,  but  soon  other  muscles  suffer.  The  atrophy  and  paralysis  of 
the  lips  interfere  with  the  pronunciation  of  labial  letters,  with  the  act  of 
blowing,  whistling  and  laughing.  Reactions  of  degeneration  appear  early. 
The  lips  being  immobile,  the  mouth  remains  open  and  the  saliva  is  con- 
tinuously dribbling.     The  facies  is  quite  characteristic  at  this  period. 


228  DISEASES    OF   MEDULLA,    PONS    AND   FOURTH  VENTRICLE 

While  the  expression  of  the  eyes  shows  total  integrity  of  intelligence,  the 
condition  of  the  mouth  gives  the  impression  of  a  stupid  and  crying  face. 
This  contrast  is  typical  of  bulbar  palsy. 

Paralysis  of  the  palate  is  manifested  by  a  change  in  the  tone  of  the 
voice;  the  latter  is  nasal.  The  food  is  then  regurgitated  through  the 
nose. 

When  the  pharynx  is  paralyzed,  the  food  is  likely  to  fall  into  the 
larynx,  as  the  deglutition  is  very  difficult.  The  patient  is  threatened 
with  suffocation.  Artificial  feeding  is  then  necessary.  The  paralysis  of 
the  larynx  increases  the  danger:  the  glottis  being  open,  the  food  easily 
falls  into  the  larynx. 

The  laryngeal  condition,  consisting  of  paralysis  of  the  vocal  cords 
and  of  all  the  muscles  innervated  by  the  recurrent  nerve,  produces  dis- 
turbances not  only  of  the  voice,  but  also  of  phonation.  The  patient  is 
unable  to  emit  even  a  sound  (aphonia). 

The  gradual  but  progressive  involvement  of  the  medulla  leads  to 
cardiac  and  pulmonary  disorders.  The  pulse  is  small,  irregular  and 
feeble;  attacks  of  syncope  are  quite  frequent.  The  least  effort  brings  on 
dyspnea.  The  patient  is  unable  to  expectorate,  to  breathe  properly: 
mucus  accumulates  in  the  bronchial  tubes.  An  ordinary  bronchitis 
becomes  thus  very  serious.     Broncho-pneumonia  develops  easily. 

Among  other  symptoms  the  condition  of  the  reflexes  should  be  men- 
tioned. Those  of  the  extremities  are  very  frequently  exaggerated. 
This  fact  shows  the  close  relation  of  bulbar  palsy  to  amyotrophic  lateral 
sclerosis. 

Course.  Duration.  Prognosis. — The  disease  is  essentially  progres- 
sive. Death  may  occur  from  three  causes:  inanition  (because  of  the 
inability  of  swallowing),  syncope  or  broncho-pneumonia.  The  latter 
may  be  of  infectious  or  gangrenous  nature  when  food  enters  the  larynx. 
The  usual  duration  of  the  disease  is  from  a  few  months  to  a  couple  of 
years. 

Diagnosis. — The  essentially  chronic  and  progresssive  course  after  an 
insidious  and  slow  onset,  the  successive  involvement  of  the  tongue,  lips, 
palate  and  masticatory  muscles,  atrophy  of  the  muscles  with  fibrillary 
contractions  and  reaction  of  degeneration  are  sufficiently  characteristic 
symptoms  for  the  diagnosis  of  bulbar  palsy. 

Palsy  of  the  palate  following  diphtheria  is  accompanied  by  difficulty 
of  swallowing  and  a  nasal  intonation  of  the  voice.  These  cases  will  be 
recognized  by  absence  of  paralysis  of  the  tongue  and  lips. 

Hemorrhage  and  softening  of  the  medulla  are  sudden  in  onset  and  the 
symptoms  are  pronounced  at  the  beginning. 


DISEASES    OF   MEDULLA,    PONS    AND    FOURTH  VENTRICLE  229 

In  Pseudo-bulbar  palsy  there  is  always  a  history  of  one  or  two  attacks 
of  apoplexy.  The  syndrome  of  labio-glosso-laryngeal  paralysis  is  usually 
established  after  the  second  attack,  but  it  has  not  the  chronic  character 
of  the  pure  bulbar  palsy.  Besides,  there  is  no  muscular  atrophy  or 
fibrillary  twitching.  The  spasmodic  laughing  and  crying,  impairment  of 
intelligence,  hemiplegic  condition  of  the  extremities  are  all  typical  of  the 
pseudo-bulbar  palsy  (see  this  chapter). 

Asthenic  bulbar  paralysis  (myasthenia  gravis)  is  recognized  by  the 
predominance  of  the  paralysis  in  the  muscles  of  mastication  and  in  the 
levator  palpebrae  (ptosis).  The  extreme  exhaustion  manifested  in  the 
muscles  upon  the  least  exertion,  the  absence  of  atrophy  and  the  special 
electrical  reactions  are  characteristic  of  myasthenia  gravis  (see  this 
chapter). 

Treatment. — Counter-irritants,  cautery  on  the  back  of  the  neck,  also 
galvanism  at  the  same  level  or  direct  electrization  of  the  affected  muscles; 
antisyphilitic  remedies,  belladonna  or  atropin  for  diminishing  the  saliva- 
tion, general  hygienic  measures  are  all  the  therapeutic  means  we  have  at 
our  command  for  a  palliative  treatment.  Unfortunately  the  disease  is 
inevitably  fatal.  When  difficulty  of  deglutition  supervenes,  artificial 
feeding  is  necessary.  In  case  of  imminent  asphyxia,  tracheotomy  should 
be  performed. 

E.  PSEUDO-BULBAR  PALSY 

This  name  is  given  to  the  syndrome  of  glosso-labio-laryngeal  paralysis 
described  in  the  preceding  chapter,  which  is  not  due  to  a  lesion  in  the 
medulla  but  in  the  brain. 

Pathology. — In  order  to  understand  the  pathological  mechanism  of 
this  symptom-group  it  is  necessary  to  recall  the  following  anatomical 
facts. 

The  muscles  of  the  tongue,  face,  lips,  pharynx  and  larynx  are  inner- 
vated by  two  systems  of  neurones.  One,  lower  or  peripheral,  connects  the 
muscles  with  the  nuclei  of  the  medulla.  The  other  connects  the  bulbar 
nuclei  with  the  cortex,  and  especially  with  the  Rolandic  operculum  (see 
Anatomy) ;  the  connecting  fibers  lie  in  the  geniculate  bundle  of  the  internal 
capsule.  A  lesion  of  the  first  neurones  will  give  place  to  the  genuine 
bulbar  palsy  (labio-glosso-laryngeal)  of  the  preceding  chapter.  A  lesion 
affecting  only  the  second  (upper  or  cortico-bulbar)  system  of  neurones 
will  result  in  a  loss  of  transmission  of  stimulation  from  the  brain  to  the 
lower  neurone,  and  we  will  have  a  pseudo-bulbar  palsy.  In  the  latter  case 
the  lesion  must  be  bilateral,  because  each  hemisphere  innervates  the 


230  DISEASES    OF   MEDULLA,    PONS   AND    EOUE.TH  VENTRICLE 

muscles  of  both  sides.  A  unilateral  lesion  of  the  cortico-bulbar  pathway 
will  therefore  fail  to  produce  the  complete  picture  of  labio-glosso-pharyn- 
geal  paralysis. 

As  to  the  nature  of  the  lesion,  it  may  be  a  hemorrhage,  softening, 
cysts  or  patches  of  sclerosis  (in  disseminated  sclerosis).  According  to 
Brissaud  the  lesion  may  occur  bilaterally  in  the  third  frontal  convolution, 
in  the  basal  ganglia,  in  the  cortex  of  one  hemisphere  and  in  the  basal 
ganglia  of  the  other.  It  may  also  occur  in  bilateral  involvement  of  the 
motor  segment  of  the  internal  capsule.  In  the  majority  of  cases  the  lesion, 
which  is  either  hemorrhage  or  softening,  is  extensive  and  involves  the 
central  ganglia  and  the  internal  capsule. 

Etiology. — Any  condition  which  is  apt  to  lead  to  a  morbid  state  of 
the  cerebral  blood  vessels  and  consequently  to  hemorrhage,  embolism 
and  thrombosis  is  the  cause  of  the  disease.  Syphilis,  arteriosclerosis, 
cardiac  diseases  are  therefore  the  chief  factors. 

Infantile  Pseudo-bulbar  Palsy  has  been  also  observed  by  Oppenheim 
and  others  in  connection  with  diplegia  (see  this  chapter) .  It  is  due  to  an 
arrested  development  or  malformation  of  the  lower  parts  of  the  central 
convolutions. 

Symptoms. — In  the  majority  of  cases  there  is  a  hsitory  at  first  of 
one  attack  of  hemiplegia  with  some  slight  disturbance  of  phonation  or 
deglutition.  Soon  a  second  apoplectiform  seizure  takes  place  and  with 
it  a  complete  picture  of  bulbar  palsy  is  established. 

All  the  symptoms  of  a  true  bulbar  palsy  will  be  observed  here.  A 
pseudo-bulbar  individual  will  therefore  present:  immobility  of  the  facies, 
stupid  expression  of  the  latter,  a  continuously  open  mouth,  dribbling 
of  the  saliva,  paralysis  of  the  muscles  of  the  cheeks,  lips,  tongue,  palate, 
of  mastication,  of  the  vocal  cords,  abolition  of  the  pharyngeal  reflex; 
finally  a  nasal  intonation  of  the  voice,  dysarthria  with  or  without  aphonia, 
dysphagia  or  complete  inability  to  swallow,  difficulty  of  respiration  with 
attacks  of  dyspnea  (see  chapter  on  Bulbar  Palsy). 

In  addition  to  these  symptoms  there  are  a  few  special  signs  charac- 
teristic of  the  pseudo-bulbar  form.  They  are:  absence  of  atrophy  and  of 
fibrillary  tremor  in  the  paralyzed  muscles,  preservation  of  normal  electrical 
reactions  and  of  the  reflexes  in  the  region  innervated  by  the  bulbar  nerves. 
To  this  may  be  added  a  unilateral  or  bilateral  hemiplegia,  frequent 
involvement  of  the  optic  nerves  (neuritis  or  atrophy),  finally  mental 
symptoms.  The  latter  consist  of  a  marked  impairment  of  memory,  of 
apathy,  confusion  and  of  dementia.  Spasmodic  attacks  of  laughing  or 
crying  are  almost  typical  of  pseudobulbar  paralysis.  The  latter  phe- 
nomenon is  generally  explained  by  an  interruption  of  inhibition  which 


DISEASES    OF   MEDULLA,   PONS    AND    FOURTH  VENTRICLE  23 1 

is  normally  trasmitted  to  the  bulbar  nucleus  of  the  facial  nerve  through 
the  cortico-bulbar  fibers. 

Course,  Duration,  Prognosis. — The  disease  is  essentially  progressive, 
but  slow  in  its  course.  It  may  be  interrupted  or  aggravated  by  slight 
apoplectiform  seizures.  The  outlook  is  grave,  as  the  termination  is 
almost  invariably  fatal.     It  may  last  many  years  before  death  ensues. 

Diagnosis. — The  sharply  defined  symptoms  enumerated  above  will  enable 
to  differentiate  this  form  from  the  true  bulbar  palsy  (see  also  the  latter). 

The  acute  bulbar  palsy  will  be  recognized  mainly  by  the  absence  of 
mental  symptoms  and  hemiplegia. 

Asthenic  Bulbar  Paralysis  (myasthenia  gravis)  presents  a  series  of 
characteristic  symptoms,  such  as  extreme  exhaustion  upon  the  least 
effort,  ptosis  and  palsy  of  other  ocular  muscles,  inability  to  hold  up  the 
head,  a  special  myasthenic  reaction  to  electricity,  etc. 

Treatment. — Antisyphilitic  medications  may  be  tried  even  in  cases 
without  a  clear  specific  history.  As  to  the  disturbance  of  respiration 
and  deglutition,  the  same  precautions  should  be  taken  as  in  the  true 
bulbar  palsy. 

F.  ASTHENIC  BULBAR  PARALYSIS   (MYASTHENIA  GRAVIS  OF  ERB  AND 

GOLDFLAM) 

Pathology. — Various  changes  in  the  central  nervous  system  have 
been  reported  by  a  number  of  observers,  but  denied  by  others,  so  that 
in  the  state  of  our  present  knowledge  there  is  nothing  definite  concerning 
the  pathology  of  the  affection.  As  in  many  cases,  the  autopsy  findings 
have  been  absolutely  negative,  the  disease  is  called:  bulbar  palsy  sine 
materia. 

It  should  be  mentioned,  however,  that  degenerative  changes  in  the 
thymus  (Weigert,  Hansemann,  GoldfLam),  tumors  in  the  lungs,  medias- 
tinum and  thymus  were  found  in  some  cases.  Degenerative  changes 
have  also  been  observed  in  the  thyroid  and  in  the  pituitary  body.  Epen- 
dymitis  of  the  aqeuduct  of  Sylvius  has  been  reported.  The  muscles 
are  very  frequently  altered:  infiltration  of  cells  between  the  fibers,  hyaline 
degeneration.  Buzzard  (Brain,  1905)  gave  the  name  "Lymphorrhagia" 
to  the  infiltrated  groups  of  lymphocytes  scattered  between  the  cells  of 
the  nuclei  in  the  medulla  and  he  considers  them  characteristic  of  myas- 
thenia gravis. 

To  explain  the  pathogenesis  of  the  affection  several  theories  have 
been  advanced.  Among  them  the  most  important  is  the  pluriglandular 
view.  According  to  it  the  altered  internal  secretions  of  various  glands 
play  an  essential  role  in  the  myasthenic  syndrome:  profound  changes 


232  DISEASES    OF   MEDULLA,    PONS    AND    FOURTH  VENTRICLE 

in  the  glands  have  been  found  in  a  number  of  cases  (see  above).  The 
pluriglandular  apparatus  has  an  effect  on  the  bulbo-spinal  motor 
system  which  is  involved  in  myasthenia  gravis.  The  disturbance  of 
this  apparatus  has  also  a  trophic  effect  on  the  muscles  involved  in 
the  disease.  Massolongo  (Riforma  Medica,  191 2)  believes  that  what- 
ever the  role  of  the  glands  with  internal  secretions  may  be,  the  clinical 
picture  of  the  affection  requires  also  a  special  inherent  predisposition 
of  an  organic  and  morphological  character  of  the  bulbo-spinal  centers. 
There  is  an  inherent  weakness  of  the  gray  motor  centers  of  the  mesence- 
phalon and  the  spinal  cord  which  produces  a  muscular  exhaustion 
following  an  exertion. 

Etiology. — The  causes  of  the  malady  are  as  little  known  as  its  path- 
ology. The  prevalent  opinion  is  in  favor  of  a  toxic  nature:  in  a  number 
of  cases  the  disease  developed  after  infectious  diseases,  also  in  instances  of 
auto-intoxication. 

Women  are  more  frequently  affected  than  men:  children  very  rarely. 

Symptoms. — After  a  brief  prodromal  period,  consisting  of  headache 
and  occipital  pain,  also  of  vertigo,  in  the  majority  of  cases  the  symptoms 
begin  with  ptosis.  The  patient  is  compelled  to  contract  the  frontal 
muscles  to  assist  the  levator  palpebras  in  raising  the  eyelids.  Very  soon 
appears  external  ophthalmoplegia,  so  that  strabismus  and  diplopia  are 
among  the  earliest  symptoms.  When  the  facial  nerve  is  paralyzed, 
both  portions  of  it  (upper  and  lower)  are  involved.  This  fact,  together 
with  the  ptosis,  gives  the  facies  a  peculiar  aspect:  the  face  is  immobile, 
without  wrinkles  and  somnolent,  the  head  is  raised  when  an  object  is 
looked  upon. 

The  muscles  of  mastication,  the  tongue,  lips  and  the  larynx  are  in  a 
paretic  condition.  Mastication,  deglutition,  phonation  are  therefore 
affected.  Gradually  the  muscles  of  the  neck  become  involved.  The 
patient  cannot  then  hold  up  his  head,  which  has  a  tendency  to  fall  for- 
ward or  backward.  This  is  quite  characteristic  of  the  disease.  In  a  still 
more  advanced  stage  the  muscles  of  the  trunk,  of  the  abdomen  and  of  the 
extremities  become  similarly  affected.  The  patient  is  unable  to  sit  up 
or  stand  up  and  has  difficulty  in  breathing;  dypncea  is  marked  on  the 
least  exertion.  In  the  shoulder  muscles  the  weakness  is  very  marked. 
The  patient  is  unable  to  hold  up  his  arms. 

In  spite  of  the  paralysis  or  paresis  of  the  muscles  of  a  more  or  less  long 
duration,  muscular  atrophy  is  extremely  rare,  but  a  very  important 
feature  of  the  affected  muscles  is  their  special  response  to  electrical  stimu- 
lation. This  is  the  so-called  myasthenic  reaction.  It  is  an  exhaustion 
reaction  and  consists  of  a  gradual  diminution  and  finally  of  a  loss  of 


DISEASES    OF   MEDULLA,    PONS    AND    FOURTH  VENTRICLE  233 

contraction,  when  a  faradic  electrical  current  is  applied  to  the  muscles. 
If  after  a  brief  rest  the  current  is  reapplied,  the  same  phenmenon  will  be 
observed.  This  reaction  is  not  observed  from  application  of  a  galvanic 
current. 

Sensations,  reflexes  and  the  function  of  the  sphincters  remain  intact. 
Subjective  aching  in  connection  with  exhaustion  is  frequently  present. 
The  reflexes  become  exhausted  by  frequent  tests.  Mentality  is  also 
preserved.  One  of  the  most  characteristic  features  of  the  disease  is  the 
remarkable  variability  in  the  paralytic  phenomena.  Thus,  for  example, 
at  a  certain  time  of  the  day,  especially  in  the  morning,  the  patient  is 
able  to  move  about  and  exercise  his  muscles,  but  he  soon  gets  exhausted. 
He  may  begin  to  speak,  but  gradually  the  voice  gets  weaker  and  finally 
complete  aphonia  sets  in.  He  may  attempt  to  whistle  or  blow  out  a 
candle;  at  first  the  acts  are  normal,  but  a  second  or  third  attempt  are 
almost  impossible. 

Course,  Duration  Prognosis. — In  the  majority  of  cases  the  onset  is 
with  the  muscles  of  the  eyes  and  the  disease  has  a  descending  course, 
but  there  are  also  observations  showing  that  it  may  begin  in  the  ex- 
tremities and  ascend.  Speaking  generally,  the  evolution  of  the  symp- 
toms is  slow  and  progressive,  although  it  may  assume  an  acute  form. 
Remissions  are  not  infrequent  and  then  the  symptoms  may  also  totally 
disappear.  The  disease  may  last  ten  or  fifteen  years.  The  prognosis 
is  grave,  as  respiratory  disturbances  are  not  infrequent.  Disturbances 
of  deglutition  are  equally  to  be  feared.  Sudden  death  may  ensue.  The 
cases  of  so-called  recovery  are  only  long  remissions. 

Diagnosis. — The  characteristic  symptoms  described  above,  viz.  the 
onset,  gradual  descending  development  of  symptoms,  characteristic 
faces,  myasthenic  reaction,  finally  the  variability  in  the  paralytic 
phenomena  are  all  sufficient  facts  to  enable  us  to  make  a  diagnosis. 

From  the  chronic  form  of  bulbar  paralysis  it  will  be  distinguished 
by  the  absence  of  atrophy,  fibrillary  contractions  and  reactions  of  de- 
generation, and  by  the  presence  of  the  exhaustion. 

Treatment.- — It  consists  mainly  of  taking  special  care  of  the  act  of 
deglutition.  The  patient  should  be  advised  to  eat  very  slowly.  As  soon 
as  difficulty  arises,  artificial  feeding  must  be  resorted  to.  Absolute  rest 
is  indispensable  in  view  of  the  extreme  exhaustion  upon  the  slightest 
effort.  The  presence  of  the  myasthenic  reaction  in  the  affected  muscles 
is  a  direct  warning  that  electricity  is  a  dangerous  procedure;  it  must  there- 
fore never  be  applied.  Adrenalin,  extract  of  thymus  and  thyroidin  have 
given  some  favorable  results  in  some  cases  (Raymond,  Buzzard).  Sezary 
(Semaine.  Med.,   1913)   suggests    that  when    adrenalin  is   given  in  ade- 


234  DISEASES    OF   MEDULLA,    PONS   AND    FOURTH  VENTRICLE 

quate  doses,  benefit  will  be  derived.  He  has  seen  cases  in  which  sub- 
cutaneous injections  of  the  whole  extract  or  ingestion  of  fresh  adrenals 
succeeded  when  the  powder  and  epinephrin  had  failed  completely. 
His  experience  demonstrates  that  certain  slow  changes  in  the  adrenals, 
alone  or  with  changes  in  other  ductless  glands,  may  induce  a  myasthenic 
syndrome.  This  justifies  adrenal  organotherapy,  supplementing  the 
adrenal  with  hypophysis  treatment. 

Among  other  remedies,  Iodides,  arsenic,  phosphorus,  iron  and  strychina 
should  be  tried. 

G.  HEMORRHAGE  AND  SOFTENING  OF  THE  MEDULLA 

Pathology. — Hemorrhages  of  the  medulla  are  rare  and  are  produced 
by  the  same  causes  as  usual  cerebral  hemorrhages.  They  frequently 
invade  the  fourth  ventricle.  Softening  is  more  frequent.  It  is  caused 
usually  by  thrombosis,  but  also  by  embolism. 

Etiology. — Traumatism  of  the  cranium,  infectious  diseases,  intoxica- 
tions, eclampsia  are  the  causes  of  hemorrhage.  Syphilis  is  a  frequent 
cause  of  thrombosis  and  cardiac  diseases  of  embolism  (see  Hemorrhage 
and  Softening  of  the  Brain). 

Symptoms. — The  general  clinical  picture  is  that  of  labio-glosso- 
laryngeal  paralysis  (see  this  chapter).  The  difference  lies  in  the  rapidity 
of  development  of  the  symptoms. 

(a)  Hemorrhage. — The  sudden  onset  may  be  followed  by  immediate 
death.  In  some  cases  the  patient  may  remain  comatose  for  hours  or 
even  days.  In  another  series  of  cases  (less  acute)  consciousness  is 
regained  and  then  the  picture  of  bulbar  palsy  becomes  evident,  viz. 
dysarthria,  difficulty  of  mastication  and  of  deglutition.  To  these  symp- 
toms are  added  paralysis  of  the  extremities,  which  presents  several 
forms.  The  most  frequent  form  is  that  of  crossed  hemiplegia,  consisting 
of  paralysis  of  the  extremities  on  the  side  opposite  to  the  lesion  and  of 
the  ninth,  tenth,  eleventh  or  twelfth  nerves  on  the  side  of  the  lesion. 
Besides  a  motor  hemiplegia,  there  may  be  present  a  hemianesthesia. 
The  symptoms  will  depend  upon  the  seat  of  the  hemorrhage. 

(b)  Softening. — The  symptoms  are  identical  to  those  of  hemorrhage. 
The  difference  lies  only  in  the  mode  of  development.  There  is  usually 
a  prodromal  period,  during  which  (especially  in  syphilitic  cases)  headache, 
somnolence,  vertigo  are  present.  The  paralytic  symptoms  of  the  cranial 
nerves  and  of  the  extremities  develop  gradually. 

Course,  Duration,  Prognosis,— In  hemorrhages  of  the  medulla,  with 
a  less  sudden  onset,  death  is  caused  by  a  difficulty  of  respiration  or  by 


DISEASES    OF   MEDULLA,    PONS   AND    FOURTH  VENTRICLE  235 

pneumonia  from  deglutition  in  the  larynx.  In  softening,  when  death 
does  not  occur  at  the  end  of  a  few  weeks,  the  symptoms  usually  show  a 
tendency  to  improve.  In  specific  cases  especially  the  prognosis  is  very 
favorable.  If  the  patient  survives  and  a  certain  number  of  motor  nuclei 
of  the  medulla  were  involved,  atrophy  with  reaction  of  degeneration  will 
remain  in  the  affected  muscles,  but  there  is  not  the  usual  symmetrical 
distribution  which  is  seen  in  typical  chronic  bulbar  paralysis. 

Diagnosis. — It  will  be  difficult  in  the  acute  cases,  but  when  the  symp- 
toms are  less  acute,  the  above  symptoms  (bulbar  palsy  associated  with 
motor  or  sensory  paralysis  of  the  extremities)  will  enable  one  to  make  a 
diagnosis.  Softening  will  be  differentiated  from  the  chronic  bulbar  palsy 
by  the  asymmetrical  distribution  of  cranial  nerve  involvement  and  para- 
lysis of  the  extremities.  Pseudo-bulbar  palsy  will  be  recognized  by  the 
history  of  apoplectic  seizures,  by  the  mental  symptoms  and  the  spas- 
modic laughing  and  crying.  Hemorrhage  will  be  differentiated  from  soft- 
ening by  the  mode  of  onset  and  evolution  of  the  symptoms. 

Treatment. — It  will  be  that  of  hemorrhage  or  softening  of  the  brain. 
Antisyphilitic  treatment  should  be  insisted  upon.  When  difficulty  of 
deglutition  makes  its  appearance,  artificial  feeding  must  be  resorted  to. 

H.  OCCLUSION  OF  THE  POSTERIOR  INFERIOR  CEREBELLAR  ARTERY. 

Among  the  earliest  typical  cases  of  thrombotic  occlusion  of  this  artery, 
verified  by  autopsy  is  that  of  Huhn  (N.  Y.  Med.  Jour.,  1897).  Spiller  has 
recently  (1908)  presented  this  subject  in  all  its  details.  The  general  picture 
of  the  disease  is  briefly  as  follows:  Sudden  onset  with  or  without  loss 
of  consciousness,  unilateral  paralysis  of  palate  and  vocal  cord;  difficulty 
of  swallowing  and  expectorating;  hemiataxia;  syringomyelic  sensory 
dissociation  on  the  opposite  side.  In  my  case  {International  Clinics, 
V.  IV)  there  was  also  right  hemiasynergia,  tendency  to  fall  to  the  right 
side,  pain  in  the  neck,  paresthesise  on  the  left  side  where  the  objective 
sensory  dissociation  was  present,  paresis  of  the  right  side  of  the  face,  re- 
traction of  the  right  eyeball,  narrowness  of  the  right  pupil.  Pathologically 
a  softening  is  found  in  one  half  of  the  medulla.  The  area  affected  lies 
between  the  spot  where  the  artery  begins  to  pass  to  the  cerebellum  and 
the  middle  of  the  nucleus  of  the  hypoglossus.  The  formatio  reticularis 
with  its  nuclei,  the  descending  root  of  the  fifth  nerve,  the  nuclei  of  the 
seventh  and  ninth  nerves,  also  the  spino-cerebellar  tract  are  all  involved. 

I  .  COMPRESSION  OF  THE  MEDULLA 

The  medulla  may  be  compressed  (a)  suddenly  by  dislocation  or 
fracture  of  the  atlas  or  axis;  (b)  slowly  by  tumors  in  the  medulla  itself  or 
in  the  neighboring  tissues  and  organs,  by  caries  or  other  diseases  of  the 


236  DISEASES    OF   MEDULLA,   PONS    AND    FOURTH  VENTRICLE 

nieghboring  bony  tissue,  by  basal  meningitis  and  quite  frequently  by 
aneurisms  of  the  basilar  or  vertebral  arteries. 

Pathology. — The  tissue  of  the  medulla  maybe  torn  in  the  sudden  cases. 
In  the  slow  cases  the  bulb  will  be  deformed  or  softened.  Secondary 
degeneration  is  the  consequence.  . 

Symptoms.- — In  cases  of  gradual  compression  pain  in  the  occiput 
and  neck  are  the  first  symptoms.  The  head  is  kept  by  the  patient  in  an 
absolute  immobility.  The  neck  is  hyperaesthetic,  active  and  passive 
movements  are  extremely  painful.  When  the  compression  extends 
downward  to  the  cervical  cord,  pain  will  be  present  also  in  the  upper 
extremities.  The  bulbar  compression  will  be  manifested  by  disturbance 
of  deglutition,  of  respiration,  of  heart  beats.  Death  may  come  on 
suddenly.  In  cases  of  aneurism  of  the  basilar  or  vertebral  arteries,  in 
addition  to  the  occipital  pain,  there  will  be  also  signs  of  cerebral  arterio- 
sclerosis, viz.  vertigo,  noise  in  the  ears,  etc.  The  characteristic  symptom 
is  the  intermittent  bulbar  manifestations  (dysarthria,  dysphagia,  dyspnea, 
arhythmia,  tachycardia).  After  they  have  existed  for  a  certain  time,  they 
gradually  improve  and  then  disappear  until  the  next  attack.  A  paralysis 
of  the  extremities  (hemiplegia  or  paraplegia)  is  almost  always  present. 
A  very  important  symptom  was  pointed  out  by  Hallopeau,  Giraudeau 
and  Killian.  The  heads  of  their  patients  were  held  in  forced  extension; 
as  soon  as  they  were  flexed,  respiratory  disturbance  occurred.  When 
compression  is  produced  by  a  tumor,  in  addition  to  the  above  symptoms, 
there  will  be  also  general  symptoms  of  cerebral  tumor  and  optic  neuritis. 

Course,  Duration,  Prognosis. — In  sudden  compression  death  is  usually 
instantaneous  from  respiratory  paralysis.  In  slow  compression  the 
patient  is  constantly  threatened  with  rupture  of  the  aneurism  or  softening 
and  destruction  of  the  tissue  of  the  medulla.  Cardiac  and  respiratory 
disorders  are  usually  fatal.  In  basal  meningitis  of  specific  nature  the 
symptoms  may  retrograde  when  under  treatment.  The  disease  may  last 
months  or  years.     Prognosis  is  grave. 

Diagnosis. — Compression  of  the  medulla  must  be  differentiated 
from  the  typical  labio-glosso-laryngeal  paralysis.  In  the  latter  there 
is  no  pain  in  the  neck,  no  motor  or  sensory  paralysis  of  the  extremities. 

Treatment.- — Antisyphilitic  treatment  should  be  tried  in  all  cases, of 
compression  of  the  medulla,  even  if  there  is  no  history  of  syphilis.  There 
is  no  medication  that  could  arrest  this  exceptionally  grave  disease. 

DISEASES  OF  THE  PONS 

The  pons  is  a  very  important  portion  of  the  brain.  It  contains  sensory 
and  motor  tracts  going  to  and  coming  from  the  brain.     Through  the 


DISEASES    OF   MEDULLA,    PONS    AND    FOURTH  VENTRICLE  237 

middle  cerebellar  peduncles  it  is  connected  with  the  cerebellum.  More- 
over a  number  of  cranial  nerves  appear  at  the  borders  of  the  pons  on 
their  way  from  their  respective  nuclei  in  the  medulla.  Consequently  the 
symptomatology  of  pontine  lesions  must  be  complex  and  vary  from  one 
case  to  another  according  to  the  part  of  the  pons  involved. 

HEMORRHAGE,  SOFTENING,  TUMORS 

Pathology.- — Hemorrhagic  foci  are  usually  located  in  the  median  line. 
They  may  spread  downward  or  forward.  They  are  rare.  Softening  is 
quite  frequent.  Thrombosis  of  the  basilar  arteiy  is  usually  found.  The 
destruction  (softening)  sometimes  occupies  the  largest  part  of  the  pons; 
sometimes  it  is  on  one  side  and  sometimes  on  both.  In  aged  people  the 
disintegration  of  the  pcntine  tissue  occurs  in  small  areas,  which  sometimes 
are  multiple.  Tumors  are  not  frequent.  Tubercles  are  the  most 
frequent.  Next  in  frequency  are  gummata.  Gliomata  may  also  occur. 
Cysts,  cancer,  abscess  are  very  rare.  (For  a  detailed  desciiption  cf  the 
effect  of  tumors  on  the  nervous  tissue,  see  Tumors  of  the  Brain.) 

Etiology. — Inflammatory  or  degenerative  conditions  of  the  arteries  will 
lead  to  their  rupture  and  produce  a  hemorrhage.  Softening  is  caused  by 
embolism  and  thrombosis;  the  formei  is  exceptional,  the  latter  is  caused 
by  a  syphilitic  or  atheromatous  degeneration  of  the  blood  vessels.  In 
cases  of  tumors,  except  gumma  and  tuberculoma,  there  is  fiequently  a 
history  of  traumatism  of  the  head. 

Symptoms,  (a)  Hemorrhage. — The  onset  is  sudden  and  loss  of 
consciousness  especially  in  large  hemorrhages  is  always  the  first  symptom. 
In  small  hemorrhages  there  may  be  no  loss  of  ccnscicusness.  When  the 
patient  has  regained  consciousness,  one  will  observe  the  following  symp- 
toms, which  are  quite  characteristic  of  a  pontine  hemorrhage:  (a)  con- 
tracture of  the  extremities  accompanied  by  generalized  convulsions,  or 
(b)  unilateral  epileptiform  convulsions;  (c)  conjugate  deviation  of  the 
eyes  and  of  the  head;  (d)  crossed  paralysis. 

The  conjugate  deviation  presents  special  features.  If  the  pontine 
lesion  is  destructive  and  leads  to  a  unilateral  paralysis,  the  eyes  will  be 
turned  toward  the  paralyzed  side.  If  it  is  irritative  and  leads  to  unilateral 
convulsions,  the  eyes  will  be  turned  away  from  the  affected  side. 

Crossed  paralysis  is  a  very  frequent  occurrence.  It  consists  of  a 
palsy  of  the  limbs  on  the  side  opposite  to  the  lesion  and  of  involvement 
of  one  or  several  cranial  nerves  on  the  side  of  the  lesion.  Besides  the 
palsy  the  symptoms  will  therefore  depend  upon  the  cranial  nerve  involved. 
When  the  lesion  is  low  down  in  the  pons,  the  seventh   nerve  will  be 


238  DISEASES    OE   MEDULLA,   PONS   AND   FOURTH  VENTRICLE 

involved;  there  will  be  paralysis  of  the  opposite  side  of  the  body  and 
facial  palsy  on  the  side  of  the  lesion.  When  the  hemorrhage  is  near  the 
upper  border  of  the  pons,  the  sixth  nerve  alcne  may  be  involved. 
There  will  be  palsy  of  the  external  rectus,  hence  internal  strabismus. 

Myosis,  dysarthria  and  dysphagia  are  quite  frequently  observed  in 
pontine  hemorrhage.     Death  occurs  from  cardiac  or  respiratory  paralysis. 

(b)  Softening. — The  onset  is  rarely  sudden,  more  frequently  slow. 
In  the  latter  case  it  is  preceded  by  prodromal  symptoms,  such  as  headache 
and  vertigo. 

The  attack  itself  is  immediately  preceded  by  paresthesia  in  the 
limbs  which  are  to  be  affected.  Then  gradually,  but  progressively,  the 
paralytic  symptoms  make  their  appearance.  Crossed  hemiplegia  is  the 
usual  result.     Dysarthria  and  dysphagia  are  quite  frequent. 

When  the  scftening  is  due  to  a  thrombosis  of  the  basilar  artery,  there 
are  also  general  symptoms  besides  the  crossed  paralysis.  They  are: 
somnolence,  potic  neuritis,  sometimes  delirium. 

(c)  Tumors. — Tumors  may  originate  in  the  pons  or  in  the  vicinity  at  the 
base,  most  frequently  in  the  ponto-cerebellar  angle.  For  the  latter  see 
page  217.  Similar  to  cerebral  turners,  pontine  neoplasms  present  general 
and  focal  symptoms.  The  first  are :  headache,  vomiting,  vertigo,  insomnia, 
optic  neuritis,  etc.  (see  Tumors  of  Brain).  The  headache  which  is  fre- 
quently occipital  may  be  accompanied  by  stiffness  of  the  neck.  Percus- 
sion of  the  occiput  and  of  the  first  cervical  spinous  process  may  be  pain- 
ful. Optic  neuritis  appears  late  or  in  the  terminal  stage  of  the  disease. 
The  focal  symptoms  are  chiefly:  crossed  or  alternate  paralysis,  which  is 
slow  in  development.  A  very  frequent  occurrence  is  subjective  pain  in 
the  paralyzed  muscles  of  the  limbs  and  face  (when  the  latter  is  involved). 
The  paralysis  may  be  unilateral  or  bilateral.  The  cranial  nerves-symp- 
toms depend  upon  the  nerve  or  nerves  affected  by  the  tumor.  In  ex- 
ceptional cases  the  neives  alone  are  involved  and  there  may  be  no  paraly- 
sis of  the  extremities.  There  are  also  cases  in  which  the  nerves  are  not 
involved  and  there  is  only  paralysis  of  the  limbs.  This  possibility  will 
occur  only  in  lesions  of  the  upper  part  of  the  pons.  When  trie  lesion  is 
in  the  middle  of  the  pons  there  may  be  a  paralysis  of  the  limbs  also  of  the 
face  on  the  side  opposite  the  lesion,  which  then  lies  above  the  decussation 
of  the  central  facial  fibers.  If  the  tumor  is  so  extensive  as  to  involve  the 
medulla,  cerebellum  or  other  neighboring  tissue,  the  symptomatology  will 
be  more  complex:  unilateral  paralysis  of  the  palate,  vocal  cords,  tongue, 
ninth,  tenth,  eleventh,  twelfth  nerves.  Polyuria,  disturbance  of  respiration 
and  of  heart  beat  are  indications  of  bulbar  involvement.  Vertigo,  tituba- 
tion  show  involvement  of  cerebellar  peduncles.     Diminution  of  central 


DISEASES    OF   MEDULLA,    PONS   AND    FOURTH  VENTRICLE  239 

visual  acuity  depends  upon  the  involvement  of  the  anterior  quadii- 
geminal  bodies;  deafness  upon  a  lesion  of  the  posterior  quadrigeminal 
bodies. 

Sensory  disturbances  are  present  mainly  in  tumors  of  the  tegmentum 
(see  Anatomy) ;  there  may  be  either  hemianaethesia  or  generalized  anaes- 
thesia. Hemianassthesia  is  quite  frequent.  Not  infrequently  it  accom- 
panies the  hemiplegia.  The  disturbance  of  sensation  is  according  to 
Marinesco  due  to  the  involvement  of 
the  mesial  fillet  and  formatio  reticularis 
in  its  ventral  part  (see  Anatomy) . 

Like  in  hemorrhage  and  softening, 
dysphagia  and  dysarthria  are  also  pres- 
ent and  even  quite  frequent  in  tumors. 
Dysphagia  is  caused  by  involvment  of 
the  medulla,  dysarthria — by  involve- 
ment of  the  central  fibers  of  the  hypo-  ^jgf  _r  .  —  ,. 
glossus.  Anarthria  implies  a  bilateral 
lesion  in  the  pons.  FlG-  So.-Tubercle  in  the  Pons. 

.  J      .     .    r  .  ,  Section  between  the  Points  of  Emer- 

Ataxia  is  frequent  in  pontine  lesions.    GENCY  0F    THE  ?TH  AND  STH  Nerves> 

It  is  usually  unilateral.      It  is  probably     {Flatau,  Jacobsohn  and  Minor.) 

due    to    the    implication    of   the  fillet 

which  produces  sensory  disturbances.     When  the  gait  and  station  are 

disturbed,  the  cerebellum  or  its  peduncles  are  probably  involved. 

Crossed  Paralysis. — From  the  foregoing  remarks  it  can  be  seen  that 
crossed  paralysis  is  a  common  feature  of  diseases  of  the  pons.  This  form 
of  paralysis  may  be  motor  and  sensory.  There  are  two  varieties  of  crossed 
paralysis :  superior  and  inferior. 

In  the  superior  crossed  paralysis  (Weber's  syndrcme)  there  is  hemi- 
plegia on  the  opposite  side  and  palsy  of  the  oculo-motor  nerve  on  the 
side  of  the  lesion.  The  hemiplegia  is  total,  viz.  face,  arm  and  leg  are  in- 
volved. The  facial  paralysis  has  the  same  characteristics  as  in  cerebral 
hemiplegia,  viz.  only  the  lower  half  of  the  face  is  paralyzed.  When  this 
form  of  crossed  paralysis  is  accompanied  by  a  tremor  of  the  paralyzed 
limbs  it  is  called:  "Benedikt's  syndrome." 

The  inferior  crossed  paralysis  (Millard-Gubler's  syndrome)  is 
characterized  by  a  paralysis  of  the  arm  and  leg  on  one  side  and  of  the 
facial  nerve,  also  sometimes  of  the  abducens  nerve,  on  the  opposite  side. 
In  certain  cases  the  hypoglossus  or  other  cranial  nerves  participate. 
When  the  root  of  the  sixth  nerve  is  involved  there  is  paralysis  of  the 
external  rectus  with  internal  strabismus,  but  when  its  nucleus  is 
affected,  the  conjugate  movements  of  the  eyes  toward  the  lesion  will 


240 


DISEASES    OF   MEDULLA,    PONS   AND    FOURTH  VENTRICLE 


be  abolished  if  the  lesion  is  destructive,  but  if  the  lesion  is  irritative,  there 
will  be  a  conjugate  convulsion  of  the  eyes  toward  the  lesion.  Other 
nerves  may  also  be  affected.  The  involvement  of  the  fifth  is  not  at  all 
rare.  Tactile  sensation  will  then  be  affected  on  the  side  of  the  lesion  in  the 
area  of  distribution  of  the  fifth,  and  in  the  limbs  on  the  opposite  side. 
The  eighth  nerve  is  rarely  involved,  but  if  it  is,  there  will  be  impairment  of 
hearing  and  subjective  sensations  of  sounds  on  the  side  of  the  lesion. 
Crossed  paralysis  may  be  exclusively  sensory  or  else  sensorimotor.  In 
the  sensory  form  there  is  anaethesia  of  the  limbs  and  trunk  on  one 
side  and  of  the  face  on  the  opposite.  The  reason  of  the  crossed  charac- 
ter of  sensory  disturbances  is  because  the  decussation  of  the  sensory  fibers 
takes  place  in  the  lower  part  of  the  medulla  (see  Anatomy). 

Prognosis. — It  is  unfavorable,  especially  in  tumors.  Only  in  syphilitic 
cases  improvement  can  be  expected. 

Diagnosis. — Pontine  hemorrhage  will  be  differentiated  from  intra- 
cranial hemorrhage  in  general  mainly  by  the  presence  of  alternate  paraly- 
sis, but  also  by  convulsions  and  hyperaesthesia  of  the  affected  limbs. 
The  conjugate  deviation  of  the  head  and  eyes  presents  a  very  important 
diagnostic  point.     Landouzy  put  down  the  following  rules : 


In  paralysis 
In  convulsions. 


Pontine  Lesion 

Eyes  turned  toward  the  para- 
lyzed side. 

Eyes  turned  toward  the  un- 
affected side. 


Cerebral  Lesion 

Eyes  turned  toward  the  non-para- 
lyzed side. 

Eye  turned  toward  the  affected 
side. 


Softening  is  recognized  by  prodromal  symptoms.  The  evolution  of 
tumors  is  slow  and  progressive. 

The  distinction  between  extra -pontine  and  intra -pontine  tumors  is  of 
great  importance,  as  surgical  intervention  depends  on  it.  The  type  of 
extra-pontine  tumors  is  tumor  of  the  ponto-cerebellar  angle. 

The  chief  characteristics  are  as  follows: 

In  tumors  of  the  angle  the  manifestations  of  intra-cranial  pressure  are 
associated  with  unilateral  palsy  (on  the  side  of  the  tumor)  of  cranial 
nerves,  especially  fifth,  seventh  and  eighth.  Hypoacousia  and  vertigo 
with  tinnitus  are  often  the  first  signs  of  localization.  To  these  are  fre- 
quently added  dysarthria,  dysphagia  and  paresis  of  associated  movements 
of  the  eyes  when  the  patient  turns  his  head  to  the  side  of  the  tumor. 
Besides,  there  are  frequently  cerebellar  manifestations  on  the  side  of  the 
tumor  (asynergia,  etc.).  The  course  is  progressive  and  gradually  new 
symptoms  appear. 

Tumor  of  cerebellopontine  angle  are  usually  encapsulated  and  benign. 


DISEASES    OF    MEDULLA,    PONS    AND    FOURTH  VENTRICLE  241 

Their  detection  is  based  chiefly  on  symptoms  of  compression  of  the  cranial 
nerves  at  the  base.  A  simple  decompressive  operation  is  indicated  in 
cases  of  a  very  precarious  estate  of  the  patient,  in  cases  of  multiple  tumors 
and  in  cases  of  cancerous  metastasis.  In  all  other  cases  a  radical  opera- 
tion is  advisable.  If  the  localization  is  made,  one  cerebellar  fossa  should  be 
opened. 

Intra-pontine  tumors  commence  often  by  associated  progressive 
paralysis  of  the  lateral  movement  of  the  eyes,  predominant  on  the  side  of 
the  lesion.  It  is  accompanied  by  motor  and  sensory  disturbances  on 
the  side  opposite  the  tumor  (crossed  paralysis.)  The  implication  of  the 
auditory  nerve  is  only  a  late  symptom. 

Treatment. — The  management  is  limited  to  the  specific  treatment. 
Surgical  intervention  is  almost  impossible  on  pontine  tumors. 

Peduncular  Syndrome. — The  cerebral  peduncles  contain  motor  and 
sensory  tracts  connecting  the  brain  with  the  medulla  and  spinal  cord. 
The  sensory  fibers  He  in  the  upper  or  dorsal  portion,  the  motor  in  the 
lower  or  ventral  portion  (see  Anatomy).  Besides,  the  superficial  origin 
of  the  third  nerves  lie  between  the  crura  near  the  upper  border  of  the  pons. 
Hemorrhage,  softening,  and  abscess  occur  very  rarely.  Tumors  are 
less  infrequent.  They  are  usually  tubercular.  The  characteristic  fea- 
ture of  a  lesion  of  the  crus  will  therefore  be  a  palsy  of  the  third  nerve  on  the 
same  side  and  hemiplegia  on  the  opposite  side.  If  the  tegmentum  is 
involved,  the  hemiplegia  will  be  accompanied  by  a  hemianaesthesia.  This 
is  Weber's  syndrome  mentioned  above.  If  the  paralyzed  limbs  are 
affected  with  tremor,  we  have  Benedict's  syndrome.  Ptosis,  external 
strabismus,  diplopia,  mydriasis — are  the  ocular  symptoms  due  to  third 
nerve  palsy.  If  the  lesion  of  the  crura  is  very  superficial  and  slight,  there 
will  be  only  third  nerve  palsy  without  hemiplegia.  If  the  tumor  invades 
both  crura  and  both  third  nerves,  there  will  be  bilateral  opthalmoplegia 
and  paralysis  of  the  four  limbs.  Sometimes  the  affected  limbs  may  pre- 
sent ataxia  (Krafft-Ebing,  Marinesco  and  others). 


16 


CHAPTER  XIX 

DISEASES  OF  THE   SPINAL  CORD 

All  the  affections  of  the  spinal  cord  may  be  classified  into  two  great 
groups:  systemic  and  diffuse.  In  the  first  the  lesion  is  confined  to  a 
certain  system  of  fibers  (tracts)  or  to  portions  of  the  cord  which  have  a 
certain  definite  function  (cells  of  anterior  cornua).  In  the  second  the 
lesion  involves  gray  and  white  matter  in  a  diffuse  manner. 

A.  SYSTEMIC  DISEASES  OF  THE  SPINAL  CORD 
I.  Tabes  Dorsalis  (Locomotor  Ataxia)  (Posterior  Sclerosis) 

The  first  intimation  of  the  existence  of  the  disease  was  given  in  a 
vague  manner  by  Romberg  in  1851,  but  it  is  mainly  Duchenne  that 
presented  a  full  and  clear  description  of  the  malady. 


Fig.  81. — Posterior  Sclerosis.     {Original.) 


Pathology. — The  chief  characteristic  lesion  of  tabes  is  a  gray  degenera- 
tion of  the  posterior  columns  and  of  the  posterior  roots.  In  advanced 
cases  this  condition  can  be  seen  even  macroscopically.  In  the  majority 
of  cases  the  disease  begins  in  the  dorso-lumbar  segment  of  the  cord. 
The  initial  lesion  is  in  the  external  portion  of  Burdach's  columns,  which 

242 


TABES 


243 


corresponds  to  the  ascending  intra-spinal  fibers  of  the  posterior  roots. 
Lissauer's  tract,  the  fibers  of  which  surround  the  end  of  the  posterior 
roots,  is  also  involved  in  the  first  stage  of  tabes.  In  the  dorsal  and 
cervical  segments  of  the  cord  Goll's  columns  are  mainly  affected.  In 
advanced  cases  the  degeneration  affects  both  tracts  (Goll's  and  Burdach's) 
through  the  entire  cord.  The  posterior  roots  participate  in  the  patholog- 
ical process,  so  much  so  that  some  authors  are  inclined  to  consider  them 
with  Nageotte  as  the  point  of  departure  of  the  affection.  Nageotte  con- 
siders radicular  neuritis  as  the  primary  lesion  of 
tabes.  He  also  calls  attention  to  a  meningitis 
at  the  level  of  the  posterior  roots,  which  is  now 
proven  to  be  a  constant  condition.  The  spinal 
ganglia  in  which  the  posterior  roots  originate 
have  been  found  altered  in  a  number  of  cases 
(atrophy  of  the  cells  and  of  their  prolongation). 
The  sensory  nerves,  the  peripheral  sensory  pro- 
longations of  the  spinal  ganglia,  are  frequently 
involved.  It  can  be  therefore  seen  that  the 
following  important  sensory  elements  are  af- 
fected in  tabes:  peripheral  sensory  nerves,  spinal 
ganglia,  posterior  roots,  posterior  meningitis, 
posteiior  columns  in  the  cord.  Otherwise 
speaking,  a  disease  of  the  ascending  sensory 
neurones  constitutes  the  characteristic  lesion 
of  tabes. 

The  histological  changes  consist  of  an  enor- 
mous diminution  or  of  a  complete  disappearance 

of    the  white  fibers.     The  fibers  that  persist 

\  Fig.    82. — Tabes.      Sta- 

are  markedly  atrophied  because  of  breaking  up    TION.    legs   Widely    Sepa- 
of  the  myelin  and  of  reduction  in    size  of  the    rated,  Feet  Everted. 
axis-cylinders.    They  are  all  substituted  by  pro- 
liferated neuroglia  and  connective  tissue.     The  blood  vessels  are  frequently 
altered:  their  walls  are  thickened  (endo-  and  peri-arteritis). 

When  the  disease  ascends  to  the  medulla,  the  roots  of  some  of  the 
cranial  nerves  become  involved.  The  optic  nerve  is  very  frequently 
affected  (gray  degeneration).  The  roots  of  the  fifth  and  eighth  nerves, 
and  the  nucleus  cf  the  tenth  nerve,  have  been  found  degenerated  in 
some  cases.  Changes  in  the  cells  of  ciliary  ganglion  have  also  been 
observed. 

The  gray  matter  is  also  sometimes  diseased.  In  the  posterior  cornua 
and  Clarke's  columns  the  short  fibers  emanating  from  the  cells  are  found 


244  TABES 

degenerated  or  atrophied.  The  cells  of  the  anterior  cornua  are  rarely 
found  altered  except  in  cases  of  tabes  associated  with  muscular  atrophy. 

The  meninges  at  the  level  of  the  posterior  roots  are  thickened  and 
present  a  certain  degree  of  leptomeningitis. 

The  pathogenesis  of  tabes  is  debatable.  In  view  of  the  fact  that 
it  is  a  disease  of  the  sensory  neurone,  it  is  difficult  at  present  to  determine 
at  what  level  of  the  entire  sensory  neurone  lies  the  point  of  departure  of 
the  pathological  process.  According  to  the  majority  the  degeneration 
of  the  posterior  roots  and  columns  follows  the  initial  alteration  of  the 
spinal  ganglia.  Others  believe  that  the  posterior  roots  open  the  disease 
and  still  others  are  inclined  to  believe  that  the  initial  lesion  is  in  the 
peripheral  sensory  nerves. 

Symptoms.  I.  Motor. — (a)  The  station  and  gait  are  characteristic 
in  a  well-developed  case  of  tabes.  The  patient  experiences  a  difficulty 
in  standing:  for  fear  of  falling  he  separates  his  legs  widely  and  even  then 
his  body  oscillates.  When  an  attempt  is  made  by  him  to  close  his  eyes 
and  bring  his  feet  close  together,  there  is  a  tendency  to  fall.  The  latter 
test  is  called  "Romberg's  sign."  In  walking  there  is  an  uncertainty, 
an  incoordination  of  movements  of  the  legs,  there  is  ataxia.  The  patient 
lifts  his  legs  high,  feet  everted,  then  throws  them  forward  and  laterally, 
and  being  unable  to  control  the  movements,  he  drops  them  on  the  ground 
with  force.  This  ataxic  gait  is  sometimes  so  pronounced  that  the  patient 
is  unable  to  make  a  few  steps  without  falling.  In  initial  stages  of  tabes 
the  ataxia  may  be  extremely  slight.  The  patient  notices  then  that  he 
is  unsteady  in  going  up  or  down  stairs  or  in  walking  in  a  dark  place. 

When  the  incoordination  is  not  marked  in  the  gait,  the  ataxia  may 
be  revealed  by  making  the  patient  to  start  to  walk  suddenly,  stop  suddenly 
or  turn  abruptly.  Also  can  it  be  detected  in  a  recumbent  position:  when 
he  is  told  to  raise  the  leg  and  touch  with  his  foot  your  hand,  this  act 
will  be  accomplished  after  several  lateral  oscillations  of  the  leg  (Fig.  82). 

When  the  upper  extremities  are  affected,  there  is  a  difficulty  in  per- 
forming fine  or  delicate  acts,  as  writing,  threading  a  needle,  etc.  When 
the  patient  is  asked  to  touch  the  tip  of  his  nose  with  the  tip  of  his  index, 
the  latter  will  make  several  movements  above  and  below  or  laterally 
before  the  nose  will  be  reached.  Another  test  for  ataxia  of  the  upper 
extremities  is  to  bring  the  tips  of  the  two  indexes  together  after  the  arms 
have  been  widely  separated.  Ataxia  in  tabes  is  due  to  loss  of  sense  of 
position  in  the  muscles  and  articulations. 

(b)  Hypotonia  of  the  Muscles. — There  is  an  unusual  flaccidity  and 
relaxation  of  the  muscles  of  the  affected  limbs,  so  that  the  latter  can  be 
easily  given  any  position.     The  entire  limb,  for  example,  can  be  extended 


TABES  245 

and  raised,  so  that  it  is  brought  in  contact  with  the  trunk.  The  knee- 
joint  may  be  overextended  and  produce  a  genu  recurvatum.  In  the 
upper  extremities  the  fingers  can  be  so  extended  as  to  form  a  right  angle 
with  the  dorsum  of  the  hand.  The  hypotonia  may  also  affect  the  muscles 
of  the  trunk,  so  that  exaggerated  movements  (passive  or  active)  can  be 
produced. 

II.  Sensory. — They  are  subjective  and  objective.  Tabetic  pain  is  very 
characteristic  and  one  of  the  earliest  subjective  symptoms.  It  is  usually 
lancinating,  knife-like,  very  sharp  and  brief.  Sometimes  it  is  burning  or 
boring,  associated  with  numbness  or  tingling.  In  the  majority  of  cases  it 
is  paroxysmal  and  may  last  from  a  fraction  of  a  minute  to  an  hour.  The 
attacks  may  be  frequent  or  appear  only  at  rare  intervals.  Between  the 
paroxysms  the  skin  may  remain  hypersensitive.  The  pain  occurs  usually 
in  the  lower  extremities  and  ulnar  side  of  the  arms.  It  frequently  in- 
vades the  viscera  and  constitutes  then  a  characteristic  symptom,  called 
"crisis."  In  gastric  crisis  the  pain  is  in  the  hypochondrium  and  an 
attack  may  be  followed  by  vomiting.  There  are  also:  vesical,  renal, 
testicular,  laryngeal  and  anal  crises. 

Instead  of  being  intermittent,  the  pain  may  remain  permanent  and 
fixed.  This  occurs  in  the  so-called  "girdle  pain,"  which  consists  of  a 
sense  of  constriction  around  the  waist  or  chest.  The  phenomenon  is 
frequent  and  typical  of  tabes.  Among  other  subjective  sensations  there 
may  be  instead  of  or  besides  pain  also  various  paraesthesiae,  as  numbness, 
tingling,  coldness,  burning,  etc.  They  may  affect  any  portion  of  the 
body,  but  more  frequently  the  lower  extremities. 

Loss  of  position  of  the  limbs  is  present  in  a  large  number  of  cases. 
The  patient  is  unable  to  tell  the  position  of  his  limbs  (lower  most  fre- 
quently) while  in  recumbent  position.  It  can  be  elicited  by  closing  his 
eyes  and  placing  one  leg  over  the  other  or  by  flexing  and  extending  a 
portion  of  a  limb. 

The  objective  sensory  disturbances  are:  anaesthesia  and  analgesia. 
The  loss  of  sense  of  pain  and  touch  is  met  with  in  tabes  over  small 
areas.  In  the  upper  extremities  it  is  usually  on  the  ulnar  side  of  the 
forearms.  In  the  lower  extremities  it  is  usually  on  the  soles  of  the  feet: 
it  is  quite  common  for  tabetics  to  have  the  impression  of  walking  on 
cotton  or  on  a  carpet.  The  loss  of  pain-sense  may  not  be  confined  to 
the  skin;  it  may  be  present  also  in  the  deeper  tissues,  as  muscles,  bones, 
joints.  Painless  fractures  or  dislocations  are  not  uncommon.  Vibration 
sense  (see  page  69)  is  also  lost.  The  following  visceral  analgesias  are 
not  infrequently  observed  in  tabes:  testicular,  epigastric,  mammary, 
tracheal,  ocular;  pressure  on  all  these  organs  provokes  no  pain.     Pressure 


246  TABES 

on  the  ulnar  nerve  (Biernacki)  and  on  the  external  popliteal  nerve 
(Bechterew)  causes  no  pain.  Abade  called  attention  to  analgesia  observed 
when  the  tendon  Achilles  is  pinched.  The  loss  of  profound  sensibility  is 
sometimes  a  very  early  sign  of  tabes. 

In  exceptional  cases  there  may  be  hyperalgesia  instead  of  analgesia. 
In  such  cases  the  slightest  touch  will  cause  unbearable  pain. 

The  temperature  sense  may  also  be  altered  to  the  same  extent  as 
touch  and  pain. 

In  some  cases  there  may  be  a  perverted,  sense,  as  for  example  a  prick 
with  a  needle  will  give  the  sense  of  burning.  Finally  there  are  cases  in 
which  the  localization  of  sensations  is  disturbed,  as  for  example  the  touch 
or  pin  prick  will  not  be  felt  by  the  patient  on  the  spot  touched,  but  on  a 
remote  part  of  the  body. 

III.  Special  Senses,  (a)  Vision. — Among  the  earliest  ocular  signs 
in  tabes  is  the  Argyll-Robertson  pupil.  It  may  precede  the  ataxia  ten  or 
fifteen  years.  It  is  characterized  by  a  sluggishness  or  loss  of  light  reflex 
and  preservation  of  the  accommodation  reflex.  It  means  that  when  a  light 
is  thrown  into  the  pupil,  the  latter  remains  immobile  instead  of  contract- 
ing. This  sign  may  be  bilateral  as  well  as  unilateral.  In  advanced  cases 
the  accommodation  reflex  may  also  be  abolished.  In  some  cases  Argyll- 
Robertson  pupil  may  be  only  transitory,  disappear  and  reappear. 

Erb  called  attention  to  the  following  phenomenon.  Normally  when 
the  skin  is  pinched,  the  pupils  contract;  in  early  tabes  this  reflex 
disappears. 

Tabetic  pupils  are  quite  characteristic.  In  the  majority  of  cases 
they  are  very  small  (myosis) .  In  some  cases  they  may  on  the  contrary 
be  much  dilated.  They  may  also  be  irregular  in  contour  and  unequal: 
one  myotic,  the  other  mydriatic. 

Paralysis  of  the  ocular  muscles  is  frequent.  It  presents  these  peculi- 
arities, that  it  does  not  affect  all  the  muscles  equally  and  that  it  is  transient 
in  the  early  stages  of  tabes.  Ptosis  is  very  frequent,  internal  or  ex- 
ternal strabismus  is  also  quite  common.  The  patient  often  complains  of 
diplopia.  The  latter  is  a  very  early  sign.  These  palsies  disappear  and 
reappear  until  the  disease  is  well  developed,  when  the  ocular  paralysis 
becomes  permanent. 

As  to  the  visual  function,  there  is  almost  always  a  diminution  of  visual 
acuity.  The  loss  of  vision  may  be  acute  or  more  frequently  chronic. 
Ophthalmoscopic  examination  reveals  an  atrophy  of  the  optic  nerve  (gray 
atrophy).  Optic  atrophy  and  Argyll- Robertson  pupil  are  early  and 
certain  signs  of  tabes,  even  when  the  other  symptoms  are  hardly  notice- 
able.    In  some  cases  the  onset  of  blindness  arrests  the  evolution  of  the 


TABES  247 

other   tabetic   symptoms.       Contraction  of   the  visual  fields  especially 
for  colors  is  not  rare. 

(b)  Audition. — When  the  trigeminal  area  becomes  involved  (see 
Pathology),  the  result  will  be  a  diminution  of  the  acuity  of  hearing 
which  may  go  to  complete  deafness,  also  various  noises  in  the  ears  and 
finally  vertigo  analogous  to  that  caused  by  ear  diseases. 

(c)  The  olfactory  and  gustatory  senses  are  occasionally  found  to  be 
disturbed. 

IV.  Reflexes. — Loss  of  patellar  tendon  reflex  (Westphal's  sign)  is 
one  of  the  earliest  symptoms  of  tabes.  At  first  the  knee-jerks  may  be 
only  diminished,  but  later  they  disappear  entirely.  The  loss  or  diminu- 
tion may  be  unilateral,  but  more  frequently  bilateral.  In  doubtful  cases 
it  is  necessary  to  have  recourse  to  the  method  of  reinforcement  (Jen- 
drassik):  the  patient  is  told  to  place  one  hand  in  the  other  and  to  pull; 
a  short  blow  over  the  patellar  tendon  will  then  enable  more  readily  to 
determine  whether  the  knee-jerk  is  present  or  absent.  The  patellar 
tendon  reflex  persists  in  the  "superior  tabes"  (see  Pathology).  Loss  of 
Achilles  tendon  reflex  is  very  frequent  in  tabes.  Its  disappearance  may 
occur  long  before  that  of  the  knee-jerk. 

The  explanation  of  the  abolition  of  the  tendon  reflexes  is  found  in  the 
breaking  up  of  the  reflex  arc,  the  sensory  portion  of  which  is  diseased. 
The  cremasteric  and  anal  are  very  frequently  involved:  they  are  dimin- 
ished or  lost. 

V.  Sphincters. — Disturbances  of  the  function  of  the  sphincter  of  the 
"bladder  appears  early  in  the  course  of  tabes.  Difficulty  of  expelling  the 
urine  is  common.  Some  patients  do  not  feel  the  necessity  of  urinating. 
In  some  cases  there  is  on  the  contrary  marked  frequency  and  imperative 
micturition.     In  the  advanced  cases  incontinence  is  the  usual  occurrence. 

The  sphincter  of  the  rectum  is  also  commonly  involved.  Constipa- 
tion, difficulty  of  defecation,  tenesmus,  incontinence — all  occur  in  tabes. 

VI.  Trophic  Disturbances.— They  affect  the  general  nutrition  and 
individual  organs  or  tissues.  The  majority  of  tabetics  are  usually  pale, 
emaciated,  with  drawn  features,  sunken  eyes. 

Among  local  dystrophies  the  arthropathies  occupy  the  first  place. 
They  were  first  described  by  Charcot  in  1868.  They  may  be  of  atrophic 
and  hypertrophic  types.  The  first  consists  of  a  more  or  less  complete 
destruction  of  the  cartilage  and  of  the  bony  extremities  of  the  joint;  the 
capsule  is  in  a  state  of  relaxation.  Extreme  mobility  of  the  joint  is  the 
consequence.  Luxation  or  subluxation  of  the  bones  occur  then  easily. 
Quite  frequently  the  synovial  membrane,  which  is  either  extremely  thin 
or  thick,  contains  an  abundant  serous  fluid.     In  the  hypertrophic  variety 


248 


TABES 


there  is  formation  of  new  bony  tissue,  of  bony  excrescences  in  the  joint. 
The  principal  characteristics  of  tabetic  arthropathies  are:  absolute 
absence  of  pain  and  a  special  oedema,  which  upon  pressure  does  not 
leave  a  depression.  As  to  the  painless  joint,  it  is  so  remarkable  that 
patients  thus  affected  will  walk  with  dislocated  joints  without  the  least 
suffering. 

The  seat  of  the  arthropathy  is  mostly  in  the  knee-joint;  next  in 
frequency  is  the  foot,  then  the  hip,  shoulder,  elbow  and  inferior  maxillary 
bone.     The    "tabetic   foot"    deserves    special    mention.     It   presents    a 


Fig.  83. — Bilateral  Arthropathy  of  the  Knee  in  Tabes.     {Glorieux  and  Van  Gehuchten.) 

thickening  of  the  internal  border  of  the  foot  and  pathologically  consists  of 
an  involvement  of  the  ligaments,  bones  and  articulations.  The  patient 
walks  on  the  inner  side  of  the  foot,  which  is  everted. 

The  bones  also  suffer  in  tabes.  There  is  rarefication  of  the  osseous 
tissue.  Spontaneous  fractures  take  place  with  great  facility:  a  very 
slight  traumatism  is  likely  to  produce  them.  They  are  also  painless. 
The  formation  of  a  callus  is  usually  normal. 

Among  other  trophic  disturbances  should  be  mentioned:  "perforating 
ulcer"  (mal  perforant).  It  consists  of  a  painless  ulceration  on  the  plantar 
surface  of  the  foot  and  especially  at  the  level  of  the  great  and  little  toes. 


TABES  249 

Muscular  atrophy  not  infrequently  occurs  in  the  lower  extremities, 
but  also  occasionally  in  the  upper.  Hemiatrophy  of  the  tongue  had  been 
observed.  Cutaneous  disturbances,  as  herpes,  gangrene,  zona,  hyper- 
hidrosis,  falling  of  the  hair  and  of  the  teeth,  brittleness  of  the  nails,  oc- 
casionally develop  in  the  course  of  tabes. 

Vasomotor  disturbances  are  frequently  observed.  They  are:  coldness 
of  the  extremities,  anemia  or  blanching  of  the  extremity  of  a  limb  ("dead 
finger"),  oedema  of  the  extremities. 

There  are  at  present  two  views  concerning  the  pathogenesis  of  the 
trophic  disturbances.  According  to  some  neurologists  the  latter  are  the 
result  of  changes  in  the  cord,  namely  in  its  anterior  cornua.  Others 
believe  in  a  peripheral  origin,  viz.  in  a  neuritis  of  the  nervous  filaments 
distributed  in  the  bones,  articulations,  etc.  As  to  the  arthropathies, 
carefully  collected  records  show  a  history  of  trauma  as  an  exciting  cause, 
(see  my  contribution  in  Med.  Record,  1909). 

VII.  Visceral  Disturbances,  (a)  Gastro-intestinal  Canal. — Apart 
from  "gastric  crises,"  mentioned  above  in  connection  with  sensory 
disturbances,  tabetics  not  infrequently  present  digestive  disturbances. 
Fournier  called  attention  to  "tabetic  anorexia,"  in  which  the  patient 
loses  the  sensation  of  hunger.  The  intestinal  trouble  consists  of  tenesmus, 
of  imperative  or  frequent  defecation.  Diarrhoea  is  quite  frequent  in  the 
preataxic  period. 

(b)  Larynx. — Laryngeal  disturbances  may  consist  of  dyspnoea,  parox- 
ysms of  cough  and  "laryngeal  crises."  The  pathogenesis  of  the  latter  is 
obscure.  However  most  frequently  a  paralysis  of  the  dilators  of  the  glottis 
is  found.  The  cause  of  the  laryngeal  disturbances  lies  probably  in  the 
involvement  of  the  ninth,  tenth  and  eleventh  nerves. 

Among  the  rare  symptoms  may  be  mentioned  the  syndrome  of 
Avelis,  viz.  hemiatrophy  of  the  velum  palatinum  associated  with  paralysis 
of  the  recurrent  nerve.  It  is  particularly  encountered  in  tabes  of  the 
upper  portion  of  the  cord.  The  paralysis  of  the  internal  branch  of  the 
eleventh  nerve  which  produces  the  hemiatrophy  of  the  palate  is  probably 
due  to  a  meningeal  involvement  of  the  root  fibers  of  that  nerve. 

(c)  Genital  Apparatus. — It  is  frequently  disturbed.  Sexual  desire 
with  orgasm  is  increased  at  the  beginning  of  tabes.  As  the  disease  pro- 
gresses, impotence  gradually  makes  its  appearance.  Sterility  is  fre- 
quent in  women. 

(d)  Blood  Vessels. — Atheromatous  changes  are  present  in  many 
cases.  This  is  probably  the  cause  of  paroxysms  of  "angina  pectoris" 
occasionally  observed  in  tabes. 

(e)  Brain. — Mental  disturbances  are  sometimes  observed,  especially 


250  TABES 

toward  the  end  of  the  malady.  It  is  mainly  cerebral  depression,  but  may 
be  also  cerebral  exaltation.  Kraepelin  speaks  of  hallucinatory  psychoses 
occurring  in  the  course  of  tabes,  but  he  does  not  affirm  that  they  are 
directly  and  exclusively  conditioned  by  tabes.  The  amaurotic  form  of 
tabes  is  particularly  associated  with  some  mental  manifestations  such  as 
delirium,  hallucinations.  Apoplectiform  seizures  may  occur.  The  cere- 
bral symptoms  occur  probably  in  those  forms  of  tabes  which  terminate 
in  paresis.  The  relation  of  both  diseases  is  remarkable.  There  are  tabe- 
tics in  whom  symptoms  of  paresis  develop  and  paretics  in  whom  symp- 
toms of  tabes  are  present.  The  involvement  of  the  posterior  columns 
in  paresis  is  almost  constant. 

Claude  (Encephale,  10  Dec,  191 2)  has  recently  brought  forward 
anatomical  facts  showing  the  existence  of  syphilitic  meningo-encephalitis 
in  tabes,  viz.  sclerotic  and  gummatous  nodules,  old  adhesions  of  the  dura, 
sclerotic  thickening  of  the  pia,  foci  of  hemorrhagic  softening  with  periph- 
eral inflammatory  reactions,  foci  of  cedematous  encephalitis.  During 
life  the  patient  presented  a  delirious  state,  confusion,  disorientation.  It 
is  evidently  a  case  of  tabes  with  localized  syphilis  of  the  central  nervous 
system,  but  not  typical  of  paresis. 

Course,  Duration  and  Termination,  Prognosis. — All  the  cases  of 
tabes  are  not  identical  to  each  other.  Generally  speaking,  three  periods 
are  observed  in  the  course  of  the  disease :  preataxic,  ataxic  and  the  terminal. 
This  division  is  by  no  means  present  in  every  case.  There  are  case  s  which 
are  ataxic  from  the  very  onset.  It  is  impossible  to  foretell  in  any  given 
case  the  mode  of  the  evolution  of  the  symptoms.  There  are  also  mild  and 
aggravated  forms.  In  certain  cases  the  symptoms  reach  a  certain  degree 
of  development  and  remain  stationary.  Others  progress  very  rapidly. 
In  the  "amaurotic  form"  the  blindness  and  optic  atrophy  may  be  the 
only  symptoms  for  years  before  other  signs  of  tabes  make  their  ap- 
pearance. 

The  course  may  be  extremely  slow  and  last  even  thirty  years,  although 
on  an  average  the  disease  lasts  ten  or  fifteen  years.  The  sphincter  dis- 
turbances hasten  the  disease.  Death  is  the  usual  termination.  Tabetics 
usually  die  in  cachexia,  but  also  from  some  intercurrent  disease  (pneu- 
monia, tuberculosis).  Sometimes  infection  from  cystitis  or  pyelonephritis 
or  else  from  a  bedsore  is  likely  to  shorten  the  patient's  life.  The  prognosis 
is  therefore  grave. 

Return  of  the  patellar  tendon  reflexes  in  the  course  of  tabes  deserves 
some  comment.  After  having  been  abolished  for  some  time,  the  knee- 
jerk  may  reappear.  This  may  happen  either  after  an  attack  of  hemiplegia, 
after  the  development  of  amaurosis,  finally  after  a  rigorous  antisyphilitic 


TABES  251 

treatment.  The  mechanism  by  which  the  return  of  the  reflex  is  done  is 
not  entirely  clear.  At  all  events,  that  the  reflex  may  return,  it  is  necessary 
that  the  anatomical  pathways  are  not  completely  destroyed,  that  the  loss 
of  the  reflex  is  not  absolute. 

Diagnosis. — The  described  seven  cardinal  symptoms  are  sufficient 
for  making  a  diagnosis  of  tabes.  The  eye  manifestations  require  special 
emphasis.  The  ocular  symptoms  enumerated  above  may  be  encountered 
in  syphilis  and  therefore  amenable  to  treatment;  consequently  the 
prognosis  is  more  favorable.  A  few  differential  points  are  necessary.  If 
ocular  symptoms  in  tabes  present  marked  fluctuation,  they  are  due  to 
syphilis  itself  and  therefore  improvement  may  be  expected  from  treat- 
ment. If  they  are  due  to  tabes,  they  are  beyond  reach.  Myosis 
and  reflex  immobility  of  the  pupil  or  dilatation  of  pupil  with 
paralysis  of  accommodation  apparatus  are  more  likely  signs  of  syphilis, 
hence  amenable  to  treatment.  Simple  atrophy  of  optic  nerves  is  very  frequent 
in  tabes,  exceptional  in  syphilis.  In  tabes  the  papilla  is  pale  from  first; 
in  syphilis  the  pallor  comes  on  gradually  after  visual  disturbances  have 
developed.  Benefit  from  specific  treatment  will  also  enable  cne  to  differen- 
tiate these  disturbances.  Syphilitic  iritis  or  perforation  of  palate  are 
never  encountered  in  tabes.  Reflex  immobility  of  pupils  is  one  of  the 
earliest  and  mcst  frequent  symptoms  of  tabes.  It  may  precede  ataxia 
for  a  number  of  years. 

Recognition  of  incipient  tabes  is  of  utmost  importance.  The  following 
manifestations  are  to  be  borne  in  mind.  Some  disorder  in  the  function 
of  the  bladder,  such  as  delay  in  micturition,  or  slight  incontinence  after 
drinking;  pain  suggestive  of  rheumatism,  neuralgia  of  the  trigeminal  and 
intercostal  nerves;  paresthesia  in  the  perineal  and  scrotal  regions;  the 
ocular  disturbances;,  absence  of  Achilles  tendon  reflex;  relaxation  of 
muscles — hypotonia  especially  of  the  knees;  tendency  to  ataxia  which 
can  be  detected  after  a  careful  examination  of  individual  muscles  of 
segments  of  the  limbs;  lack  of  perception  of  vibration  of  the  tuning  fork 
when  applied  to  the  tibia;  gastric  crises — consisting  at  first  of  retching, 
salivation  and  hiccough.  In  spite  of  the  facility  with  which  a  diag- 
nosis of  tabes  can  be  made,  in  some  cases  difficulties  may  be  en- 
countered. The  following  are  the  affections  from  which  tabes  should  be 
differentiated. 

Multiple  neuritis  presents  in  common  with  tabes  pain,  loss  of  knee- 
jerks,  incoordination,  Romberg's  sign,  but  the  symptoms  absent  in  tabes 
are:  tenderness  of  the  nerve- trunks  and  sensory  disturbances,  which 
follow  the  distribution  of  the  nerves.  Finally  Argyll-Robertson  pupil  is 
never  present  in  neuritis. 


252  TABES 

Multiple  sclerosis  can  be  recognized  by  nystagmus,  intention  tremor 
and  characteristic  staccato  speech. 

Friedreich's  ataxia  presents  like  tabes  loss  of  knee-jerks,  but  it  is 
differentiated  by  the  existence  of  nystagmus  and  special  speech  and  by 
absence  of  pain. 

Cerebellar  diseases  (tumor,  hemorrhage,  etc.)  simulate  sometimes 
tabes,  but  the  difficulty  in  walking  is  net  like  a  tabetic  ataxia,  but  a  tituba- 
tion.  Moreover  cerebellar  asynergia  does  not  exist  in  tabes.  Finally 
absence  of  pain  and  cf  visceral  disturbances  are  in  favor  of  a  cerebellar 
disease. 

Recent  researches  have  demonstrated  that  in  the  course  of  tabes  a 
lymphocytosis  takes  place  in  the  cerebro-spinal  fluid  of  the  majority  of 
cases.  Wasserman  reaction  in  the  cerebro-spinal  fluid  is  almost  invariably 
positive  in  untreated  cases.  These  two  factors  are  certainly  a  valuable 
addition  to  the  semeiology  of  tabes  in  doubtful  cases. 

Etiology. — Syphilis  plays  the  most  important  if  not  the  exclusive  role 
in  the  genesis  of  tabes.  According  to  Fournier,  93  per  cent,  of  tabetics 
present  a  clear  history  of  syphilis.  Erb  and  Oppenheim  observed  patients 
personally  free  from  specific  infection,  but  whose  parents  had  had  syphilis. 
With  the  discovery  of  Wasserman  reaction  the  syphilitic  origin  of  tabes  is 
finally  settled. 

The  posterior  sclerosis  is  produced  not  directly  by  syphilis,  as  the 
disease  develops  many  years  after  the  initial  syphilitic  lesion.  Tabes 
belongs  therefore  to  Founder's  "parasyphilitic  affections."  As  to  other 
causes  mentioned  by  some  writers,  they  are:  cold,  exertion,  excesses  and 
traumatism.  In  my  opinion  they  may  be  considered  only  as  predisposing 
factors. 

The  age  at  which  tabes  occurs  is  usually  between  thirty  and  forty, 
although  juvenile  tabes  is  not  unknown.  I  have  seen  as  young  as  eighteen 
and  as  old  as  sixty-five.  Tabes  is  infrequent  in  women.  It  is  rare  in 
negroes. 

Treatment. — In  view  of  the  syphilitic  etiology  of  tabes,  mercury 
and  iodides  have  been  recommended,  but  it  is  doubtful  if  these  drugs  are 
capable  of  altering  a  sclerosis  of  the  posterior  columns.  However  as 
some  favorable  results  have  been  obtained  by  competent  neurologists,  it 
is  advisable  to  apply  this  treatment  in  every  case  of  tabes. 

Mercurials  should  be  given  first.  Inunctions  or  hypodermic  injec- 
tions are  preferable  to  internal  administration.  Precautions  must  be 
taken  against  mercurial  intoxication.  The  patient  should  be  instructed 
to  observe  himself  closely.  In  case  marked  salivation,  swelling  or  sore- 
ness of  the  gums  and  of  the  tongue,  pain  in  the  abdomen,  diarrhoea,  ele- 


TABES  253 

vation  of  temperature  are  noticed,  mercury  should  be  discontinued  with- 
out delay.  In  one  case  under  my  care  the  patient  failed  to  report  to  me 
in  spite  of  my  urgent  warning  and  kept  on  using  the  mercurial  inunctions; 
only  a  few  doses  brought  on  such  an  enormous  swelling  of  the  tongue 
that  a  tracheotomy  had  to  be  performed  for  relief  from  threatening  suffo- 
cation. In  order  to  obtain  the  full  effect  of  mercury,  it  should  be  admin- 
istered as  long  as  there  are  no- signs  of  intolerance.  As  to  the  daily  dose 
of  mercury,  it  is  advisable  to  begin  with  a  small  quantity,  say  a  half  of 
a  dram,  twice  a  day  (in  an  adult).  At  the  end  of  three  or  four  days 
the  dose  can  be  increased  to  a  dram,  if  there  is  complete  tolerance. 
After  a  sufficiently  long  trial  (say  two  or  three  weeks)  mercury  should 
be  substituted  by  iodides.  Sodium  iodide  gives  the  same  therapeutic  re- 
,  suits  as  potassium  iodide  and  has  this  advantage  over  the  latter,  that  it 
is  less  apt  to  disturb  the  stomach.  Its  initial  dose  should  also  be  small, 
say  10  minims  of  a  saturated  solution  three  times  a  day.  At  first  the 
dose  should  be  increased  five  minims  every  three  days  until  the  dose  of 
50  minims  is  reached.  Then  the  increase  should  be  very  slow:  about 
one  or  two  minims  every  other  day.  The  drug  can  be  continued  indefi- 
nitely until  intolerance  shows  itself.  The  latter  is  manifested  by  loss  of 
appetite,  digestive  disturbances  (pain,  eructation)  and  diarrhoea.  The 
question  of  intolerance  is  a  relative  one.  I  have  had  patients  who  have 
been  unable  to  take  more  than  five  minims  t.  i.  d.  and  patients  that 
could  take  500  minims  t.  i.  d. 

Arsenical  preparations  have  been  recently  brought  forward.  The 
newly  discovered  Salvarsan  by  Ehrlich  and  Hatta,  although  a  very  valu- 
able drug  in  primary  and  secondary  stages  of  syphilis  is  practically 
valueless  in  tabes.  It  may  in  some  cases  improve  the  general  nutri- 
tion but  it  has  very  little  or  no  effect  upon  the  course  of  the  disease. 
This  subject  will  be  discussed  fully  in  chapter  on  Cerebro-spinal  syphilis. 
Cacodylate  of  sodium,  another  arsenical  preparation,  administered  hypo- 
dermically  has  also  a  tendency  to  improve  the  patient's  general  health, 
but  it  has  no  effect  on  tabes  itself. 

Among  other  drugs  used  in  treatment  of  tabes  can  be  mentioned: 
chloride  of  gold,  nitrate  of  silver  and  ergot.  The  latter  has  been  recom- 
mended by  Charcot  to  combat  the  genito-urinary  disorder.  The  special 
symptoms  of  which  the  tabetics  complain  can  be  combated  by  usual 
medicaments.  But  there  is  one  manifestation  which  requires  special  at- 
tention. This  is  "  gastric  crises."  Because  of  the  intensity  of  the  pain, 
of  frequent  vomiting  and  the  asthenia  which  the  latter  produces,  gastric 
crises  are  the  most  distressing  manifestations  of  tabes. 

The  crisis  is  the  result  of  motor  and  secretory  reflex    phenomena 


254  TABES 

secondary  to  a  hyperesthesia  of  the  gastric  mucosa.  The  latter  is  con- 
trolled by  the  pneumogastric  and  especially  by  the  sympathetic.  These 
two  nerves  send  filaments  to  the  major  splanchnic  through  the  solar 
plexus  and  from  there  through  the  rami  communicantes  they  reach  the 
posterior  roots  of  the  sixth,  seventh,  eighth  and  ninth  dorsal  pairs.  To 
decrease  the  irritability  of  these  nerves  local  applications  have  been  ad- 
vised, such  as  ice,  cauterization,  spray  of  ethyl  chloride  over  the  region  of 
the  stomach ;  internally  chloroform,  opium,  etc.  They  all  may  occasionally 
give  temporary  relief. 

Surgical  procedures  have  been  more  successful.  Lumbar  puncture 
with  evacuation  of  10  or  30  c.c.  gave  in  some  cases  considerable  relief, 
but  still  better  results  were  obtained  when  the  puncture  was  followed  by 
some  anesthetizing  fluid  such  as  cocain,  eucain,  novocain,  stovain  in  the 
amount  of  1  c.c.  of  1 :  10  solution.  Epidural  injections  have  given  similar 
results  to  Konig  and  others.  None  of  these  fluids  could  be  considered 
absolutely  safe,  as  accidents,  such  as  paralyses  or  respiratory  disturbances 
have  been  observed.  Besides,  not  in  all  cases  improvement  has  been 
noticed.  A  more  satisfactory  drug  for  intraspinal  injection  is  magnesium 
sulphate.  Its  dose  is  1  c.c.  to  each  25  pounds  of  body  weight;  jthe  solu- 
tion is  25  per  cent.  With  this  drug  fewer  accidents  and  more  frequent 
improvements  have  been  observed. 

Recently  operations  of  Foerster  and  Franke  came  to  the  front.  Both 
have  met  with  successes  and  failures.  Both  have  for  object  to  interrupt 
the  sympathetic  pathway  of  the  gastric  sensibility.  Foerster  attacks  it 
at  the  level  of  the  posterior  roots  of  the  sixth,  seventh,  eighth,  ninth  and 
tenth  thoracic  nerves.  He  cuts  these  roots  on  both  sides.  The  opera- 
tion is  a  complex  one  and  the  mortality  is  quite  high.  However,  excellent 
results  have  been  obtained  in  some  cases.  Recurrences  of  pain  have  been 
also  observed. 

The  operation  of  Franke  is  decidedly  less  complex  and  attended  by 
a  lesser  mortality,  but  the  success  is  not  as  frequent  as  in  Foerster's 
operation.  Franke  attacks  the  sympathetic  pathway  by  tearing  out  the 
central  ends  of  the  intercostal  nerves  corresponding  to  the  roots  utilized 
in  Foerster's  operation.  Both  operations  gave  favorable  and  unfavor- 
able results.  As  the  latter  is  considerably  less  serious  than  the  first,  it 
may  be  tried  at  first,  and  when  no  results,  Foerster's  operation  may  be 
undertaken. 

Sicard  (Presse  Medicale,  191 2)  has  recently  devised  a  new  procedure 
which  seemingly  gave  him  very  satisfactory  results.  He  advises  to  pene- 
trate after  a  laminectomy  into  the  epidural  space  and  without  opening 
the   dura   tear  out   the  spinal  ganglia.     It  is    therefore   an  extradural 


TABES  255 

ganglionectomy.  It  has  the  advantage  of  avoiding  an  injury  to  the 
dura  and  consequently  there  is  no  loss  of  cerebro-spinal  fluid.  The 
number  of  spinal  ganglia  to  be  removed  on  both  sides  as  well  as  the 
number  of  roots  to  be  taken  into  consideration  for  the  operation  are  to 
be  determined  according  to  the  seat  of  the  painful  phenomena. 

Finally  injections  of  alcohol  or  stovain  into  the  point  of  emergency 
of  the  intercostal  nerves  (Koenig)  have  been  tried.  Exsner  (Wiener  klin. 
Wchn.,  191 2)  suggested  a  method  of  treating  gastric  crises  of  tabes  by 
severing  the  vagus  nerve  on  both  sides.  He  found  the  nerve  frequently 
diseased. 

Stretching  the  solar  plexus  has  also  been  recommended  and  favorable 
results  have  been  reported  by  some  surgeons.  (Audibert.  These  de 
Lyon,  1912.) 

External  Treatment  of  tabes  has  been  applied  in  various  forms,  but 
the  majority  of  them  present  only  a  historical  interest.  Suspension, 
for  example,  or  forced  flexion  of  the  spinal  column,  also  elongation  of 
nerves,  were  imagined  with  the  object  of  improving  ataxia  and  of  reliev- 
ing pain.  Massage  is  a  good  procedure  for  keeping  up  the  nutrition  of 
the  muscles;  so  it  is  with  electricity.  Among  all  the  mechanical  means 
the  "systematic  exercises"  of  the  ataxic  limbs  have  proved  to  be  most 
valuable.  Frenkel,  of  Hayden,  made  a  special  study  of  them  and  brought 
them  up  to  a  regular  method  based  upon  the  exact  knowledge  of  physio- 
logic functions  of  various  muscles.  It  consists  of  slow  exercises  of  vari- 
ous segments  of  the  limbs.  The  patient  is  taught  to  sit  down,  get  up, 
bring  his  limbs  together,  raise  one  limb  at  the  time,  walk  in  a  certain  direc- 
tion, etc.  By  this  procedure,  kept  up  persistently  for  weeks  and  months, 
patients  learn  how  to  use  their  limbs  and  may  become  so  skillful  that  they 
may  dispense  with  their  canes  or  crutches. 

In  treatment  of  tabes  general  hygienic  measures  should  not  be  neg- 
lected. Stimulants  should  be  avoided.  Good  nutritious  food  and  fresh 
air  are  indispensable.  Hydrotherapy  in  the  form  of  a  Scotch  douche 
or  baths  is  a  good  adjuvant  to  other  procedures.  Special  care  should  be 
taken  of  the  sphincters.  In  case  of  incontinence  of  urine  the  patient 
must  be  provided  with  a  special  urinal.  In  case  of  retention  catheteriza- 
tion is  necessary.  In  case  of  cystitis  the  bladder  should  be  washed  out 
with  some  mild  antiseptic,  such  as  boric  acid,  and  urotropin  adminis- 
tered internally.  Bed-sores  must  be  taken  special  care  of.  Infection 
may  originate  from  this  particular  source  as  well  as  from  the  bladder, 
and  hasten  death. 

Fatigue  must  be  avoided,  although  mild  outdoor  exercises  are  advisable. 

Anorexia  or  other  dyspeptic  symptoms  must  be  promptly  remedied, 


256  SPASTIC   PARAPLEGIA 

as  it  is  very  important  to  have  the  patient  to  assimilate  abundant  and 
nutritious  food.  In  recent  years  treatment  with  X-rays  has  been  tried. 
Favorable  results  are  being  reported.  It  is  claimed  that  the  pain  in  the 
limbs  and  crises  are  particularly  benefited.  Incoordination  may  also 
improve.  The  exposure  to  the  rays  should  last  fifteen  minutes  and  made 
at  the  level  of  the  dorso-lumbar  region.  Twenty  or  thirty  seances  are 
necessary  to  obtain  improvement. 

Juvenile  Tabes. — Tabes  has  been  observed  in  very  young  individuals 
and  even  in  children  of  nine  years  of  age.  Hereditary  syphilis  can  always 
be  traced  in  the  family  history.  Wassermann  reaction  is  positive  in  a 
much  larger  proportion  than  in  adults.  Atrophy  of  the  optic  nerve  is 
according  to  Barkan  (Wien.  klin.  wchn.,  March,  1913)  found  in  80  per 
cent,  of  all  the  cases  on  record.  The  Romberg  sign  and  ataxia  are  less 
frequent  in  children.  Mercury  does  not  arrest  the  progress  of  tabes. 
Only  one  case  of  juvenile  tabes  is  on  record  with  necropsy. 

II.  Spastic  Paraplegia  (Primary  Lateral  Sclerosis) 

Pathology. — It  consists  of  a  primary  degeneration  and  sclerosis  of 
the  pyramidal  bundles  in  the  cord.  In  the  majority  of  cases  recorded 
the  lesion  overstepped  the  boundary  and  some  changes  were  found 
also  in  GolPs  columns,  occasionally  also  in  the  direct  cerebellar  tract. 
In  a  very  few  cases  the  lesion  was  strictly  confined  to  the  lateral  columns. 
In  my  case  (New  York  M.  J.,  191 2)  only  the  pyramidal  tract  was  involved; 
the  lesion  extended  to  the  medulla  at  the  level  of  decussation.  The 
disease  is  rare. 

Etiology. — Except  the  family  form  of  spastic  paralysis,  which  will 
soon  be  described,  nothing  definite  is  known  in  regard  to  the  causes  of  the 
affection.  As  the  disease  is  very  rare,  very  few  cases  are  on  record. 
The  disease  probably  belongs  to  the  category  of  congenital  abnormalities 
of  development  of  the  central  nervous  system.  Syphilis  may  produce 
the  picture  of  lateral  sclerosis. 

Symptoms. — The  disease  is-  characterized  by  a  slow  development  of 
spasticity  with  loss  of  power  in  the  lower  extremities.  The  gait  gradually 
becomes  more  and  more  difficult  and  the  patient  is  unable  to  raise  his 
limbs  off  the  ground:  his  feet  scrape  the  floor.  Upon  passive  movements 
great  resistance  is  felt.  The  limbs  are  rigid.  The  abnormal  reflexes 
which  are  usually  found  whenever  the  motor  tract  is  involved  (see  hemi- 
plegia) are  all  present  here,  viz.  increased  knee-jerks  and  Achilles'  tendon 
reflex,  ankle-clonus,  Babinski's  phenomenon,  Oppenheim's  and  paradox- 
ical reflexes. 


SPASTIC   PARAPLEGIA 


257 


The  sensations,  the  state  of  the  bladder  and  rectum,  the  consistency 
and  electrical  reactions  of  the  affected  muscles  are  all  normal. 

Family  Spastic  Paralysis. — The  spastic  paraplegia  just  described  may 
sometimes  be  found  in  several  members  of  the  same  family.  Strumpell 
was  the  first  to  call  attention  to  this  occurrence.  In  the  majority  of  cases 
the  male  members  are  more  frequently  affected  than  the  female.  The 
disease  usually  appears  between  fifteen  and  thirty  years  of  age.  In  one 
family  that  came  under  my  observation  there  were  seven  members  affected 
with  spastic  paralysis:  four  of  them  began  to  show  the  spasticity  at  the 
age  of  three;  six  of  them  were  males  and  one  a  female.  In  families  thus 
affected  there  is  usually  present  some  degenerative  basis;  either  insanities, 
various  neuroses  or  alcoholism,  consanguinity,  syphilis.     The  symptoms 


Fig.  84.    Lateral  Sclerosis.     (Original.) 


are  the  same  as  described  above.  The  feet  are  frequently  deformed  and 
may  present  all  forms  of  deformation,  most  frequently  equinovarus. 
The  lower  extremities  are  mainly  involved.  The  upper  ones  are  rarely 
affected. 

In  some  cases  this  type  of  the  disease  remains  unchanged  until  the 
end  of  the  patient's  life,  but  in  the  majority  of  cases,  years  after  the 
onset,  new  symptoms  develop  and  usually  the  symptomatology  of  multiple 
sclerosis  becomes  apparent.  The  involvement  of  upper  extremities, 
the  staccato  speech,  the  intention  tremor,  nystagmus,  optic  atrophy — all 
are  typical  of  the  latter  affection.  It  is  interesting  to  note  that  the 
intelligence  is  invariably  intact. 

Closely  allied  to  family  spastic  paralysis  stands  Little's  disease,  which 
17 


258  SPASTIC   PARAPLEGIA 

is  a  cerebro-spinal  involvement  of  the  pyramidal  tracts,  but  the  cerebral 
symptoms  which  are  usually  present  from  the  very  beginning  make  it  a 
disease  apart;  they  are:  speech  disturbances,  strabismus,  athetcid  move- 
ments, epilepsy  (see  Cerebral  Palsies  of  Childhood). 

Prognosis. — In  the  pure  type  of  spastic  paraplegia  (which  is  rare)  and 
in  the  family  type  the  prognosis  is  unfavorable.  The  disease  is  pro- 
gressive. Complete  paralysis  with  marked  contractures  is  inevitable. 
Death  is  rarely  the  result  of  the  malady  itself,  but  mostly  of  some  inter- 
current disease,  as  infection  or  tuberculosis. 

Diagnosis. — As  spastic  paralysis  may  be  the  ultimate  consequence 
of  myelitis,  of  amyotrophic  lateral  sclerosis,  compression  of  .the  cord, 
syringomyelia,  Friedreich's  disease,  multiple  sclerosis,  the  diagnosis  of 
spastic  paraplegia  as  a  primary  affection  should  be  made  very  cautiously. 
The  disease,  as  said  above,  is  very  rare  and  consequently  can  be  diagnosed 
only  after  the  special  symptoms  of  the  other  diseases  have  been  eliminated. 
The  occurrence  of  the  spastic  paralysis  in  several  members  of  the  same 
family  is  the  most  important  characteristic  feature  of  the  disease. 

Treatment. — It  is  the  same  as  in  Little's  disease.  See  methods  of 
Foerster,  Schwab  and  Allison;  also  of  S  toff  el  (pages  122  and  123). 

PARAPLEGIA  OF  THE  AGED 

Paraplegia  of  the  aged  has  its  orgin  in  vascular  sclerosis  in  the  brain 
or  in  the  spinal  cord  producing  a  general  disturbance  in  circulation  and 
nutrition  of  the  central  nervous  system. 

Paraplegia  of  cerebral  origin  is  caused  by  "lacunar  softening,"  a  con- 
dition so  frequently  met  with  in  senile  brains  (Marie,  Ferrand  and  others) . 
It  consists  of  perivascular  degeneration  of  brain  tissue  (see  page  83)  in 
the  basal  ganglia  irregularly  distributed,  and  of  a  descending  secondary 
degeneration  in  the  pyramidal  tracts.  The  posterior  columns  of  the  cord 
are  also  irregularly  degenerated.  The  symptoms  begin  insidiously  with- 
out apoplectic  features.  They  consist  of  a  spastic  feebleness  in  the 
lower  extremities  (the  upper  limbs  are  but  very  slightly  involved)  which 
gradually  develops  into  a  total  spastic  paraplegia  with  or  without  sphincter 
involvement.  Finally  dementia,  bed-sores  and  dysarthria  make  their 
appearance.  There  is  usually  an  inequality  of  the  paretic  phenomena 
on  both  sides.  The  contractures,  if  present,  are  never  so  marked  and 
constant  as  in  the  following  type. 

In  Paraplegia  of  spinal  origin  there  is  a  disseminated  and  diffuse 
sclerosis  in  the  posterior  columns  but  especially  in  the  lateral  columns. 
The  sclerosis  is  irregularly  distributed  at  different  levels  of  the  cord. 
Clinically   the  paretic  condition    is  symmetrically  distributed  in  both 


ATAXIC   PARAPLEGIA 


259 


lower  limbs.  The  onset  is,  like  in  the  first  variety,  insidious.  The  evo- 
lution of  the  symptoms  is  longer.  There  is  frequently  pain  which  is 
continuous  and  occasionally  paroxysmal.  The  sense  of  position  is  fre- 
quently perverted.  When  the  patient  walks,  his  legs  are  widely  sepa- 
rated. There  is  an  uncertainty  in  movements  and  sometimes  the  legs 
suddenly  give  way.  The  tendon  reflexes  are  exaggerated.  The  spasticity 
gradually  increases;  contractures  in  flexion-position  develop.  Finally  ap- 
pear sphincter  disturbances,  general  emaciation  and  tendon  retraction. 
Bed-sores  may  precipitate  the  fatal  termination.  The  preservation  of 
the  mental  faculties  is  a  distinguishing  feature.  The  latter  together  with 
the  slow  course  and  the  symmetrical  distribution  of  the  spastic  and 
paralytic  phenomena  will  enable  one  to  differentiate  it  from  the  cerebral 
form. 

III.  Ataxic  Paraplegia  (Postero-Lateral  or  Combined  Sclerosis) 

Pathology. — The  lesion  consists  of  a  degeneration  and  sclerosis  of  the 
posterior  and  lateral  columns.  In  the  posterior  portion  of  the  cord  GolPs 
columns  are  mainly  affected.     In  the  lateral  portion  of  the  cord  the  crossed 


Fig.  85. — Combined  Sclerosis.     {Original.) 

pyramidal  tract  is  chiefly  and  always  involved,  but  quite  frequently  the 
direct  cerebellar  tract  and  occasionally  Gowers'  bundle  are  also  diseased. 
The  neuroglia  tissue  is  proliferated.  Changes  are  also  observed  in  the 
cells  of  Clarke's  columns. 

Symptoms. — From  the  pathological  condition  it  can  be  seen  that 
the  symptoms  of  tabes  and  spastic  paraplegia  should  be  expected.  When 
the  posterior  columns  are  more  and  earlier  involved  than  the  lateral,  the 


260  ATAXIC   PARAPLEGIA 

predominant  symptoms  will  be  those  of  tabes.  In  case  the  crossed 
pyramidal  tract  is  mainly  affected,  the  chief  symptoms  will  be  those  of 
spastic  paraplegia.  In  the  majority  of  cases  the  tabetic  symptoms  are 
few  in  number  and  the  following  are  the  manifestations  of  the  disease: 
ataxia,  Romberg's  sign,  spasticity  and  weakness  of  the  lower  extremities, 
spastic  gait,  increased  knee-jerks,  ankle-clonus,  Babinski's  sign,  Oppen- 
heim's  and  the  paradoxical  reflexes.  The  sensations,  if  they  are  altered, 
present  some  objective  changes.  At  first  there  is  a  slight  diminution  of 
sensations  to  all  forms.  Later  in  a  fully  developed  case  a  more  or  less 
complete  loss  of  sensations  is  observed.  The  anaesthesia  is  confined  to  the 
lower  extremities  and  may  reach  the  abdomen.  Paraesthesia  in  the  lower 
limbs,  such  as  numbness,  tingling  and  others,  are  an  early  symptom. 
They  may  persist  during  the  entire  course  of  the  affection.  The  main 
tabetic  symptoms :  pain,  bladder  disturbance  and  eye  changes  are  extremely 
rare.  Loss  of  control  of  the  bladder  sphincter  occurs  only  in  the  terminal 
period  when  bed-sores  and  poor  general  health  indicate  the  approaching 
end. 

In  Anemia  or  Pernicious  Anemia  the  clinical  picture  is  somewhat 
different  from  the  usual  type  of  ataxic  paraplegia.  Sensory  disturbances 
appear  at  the  beginning;  they  consist  of  paresthesia,  diminished  sensations 
and  sometimes  lancinating  pains.  In  my  case  (New  York  M.  J.,  1909) 
there  was  a  distinct  syringomyelic  sensory  dissociation.  Muscular 
weakness  and  paresis  affect  the  upper  extremities  as  well  as  the  lower. 
The  ataxia  is  more  of  a  cerebellar  type  (see  Cerebellum)  than  of  a  tabetic 
character. 

In  pernicious  anemia  degeneration  is  found  not  only  in  the  posterior 
and  lateral  columns,  but  also  in  other  portions  of  the  cord.  In  my  case 
(loc.  cit.)  the  condition  was  diffuse  and  involved  also  Gower's  tract. 

In  chronic  intoxication  with  Ergot  and  in  Pellagra  degeneration  of 
the  posterior  and  lateral  columns  sometimes  occurs.  In  ergotism — the 
symptoms  are  more  of  tabetic  nature,  while  in  pellagra  more  of  the  type 
of  spastic  paraplegia. 

Course,  Prognosis.- — The  outlook  is  unfavorable.  The  disease  runs  a 
rapid  course:  from  a  few  months  to  two  years. 

Diagnosis. — When  the  tabetic  symptoms  are  marked,  the  disease  is 
differentiated  from  true  tabes-  by  the  absence  of  the  chief  symptoms  of 
the  latter  (Argyll-Robertson's  pupil,  optic  atrophy,  bladder  disturb- 
ances, etc.)  and  by  the  presence  of  spasticity  with  the  abnormal  reflexes 
mentioned  above. 

From  Friedreich's  disease,  which  is  also  a  postero-lateral  sclerosis,  it 
will  be  differentiated  by  the  absence  of  the  chief  symptoms  of  the  first, 


Friedreich's  ataxia  261 

viz.  nystagmus,  disturbed  speech,  choreiform  movements,  early  onset  and 
family  character  of  the  disease. 

Multiple  sclerosis  will  be  recognized  by  intention  tremor,  staccato 
speech,  nystagmus. 

Transverse  myelitis  in  its  chronic  form  assumes  frequently  the  form 
of  spastic  or  ataxic  paraplegia,  but  in  the  former  the  sensory  and  trophic 
disturbances,  sphincter  involvement  and  the  evolution  of  the  symptoms 
will  easily  reveal  the  true  nature  of  the  disease. 

Etiology. — Syphilis  may  be  the  cause.  The  age  at  which  the  disease 
occurs  is  between  twenty  and  forty.  In  the  course  of  anemia,  pernicious 
anemia,  pellagra,  ataxic  paraplegia  is  sometimes  observed.  As  the  blood 
vessels  are  frequently  found  intact,  the  toxic  element  which  is  probably 
at  work  in  pernicious  anemia  follows  the  route  of  the  nerve  fibers  in  the 
cord  and  produces  a  degeneration.  In  the  absence  of  the  above  causes,  a 
congenital  weakness  of  the  sensory  and  motor  neurones  of  the  cord  is 
probably  the  true  nature  of  the  disease. 

Treatment. — Antisyphilitic  drugs  (mercury  and  iodides,  also  salvarsan) 
should  be  always  tried.  In  cases  of  anemia,  appropriate  remedies  (iron, 
arsenic)  should  be  administered.  As  to  the  ataxia  and  spasticity,  they  will 
be  relieved  by  the  same  means  as  advised  in  tabes  and  spastic  paraplegia. 

IV.  Friedreich's  Disease  (Hereditary  Ataxia) 

This  affection  was  described  first  by  Friedreich  in  1861  as  a  peculiar 
variety  of  tabes  occurring  in  childhood  and  having  a  hereditary  character. 

Pathology. — The  spinal  cord  is  diminished  in  size  and  very  slender. 
The  changes  affect  the  white  and  the  gray  matter,  but  it  is  the  first  that 
is  particularly  affected.  The  most  pronounced  degeneration  is  in  Goll's 
columns,  which  are  involved  through  the  entire  cord.  Burdach's  tract  is 
affected  mainly  in  the  lumbar  region  and  only  a  small  portion  of  it  is 
sclerosed.  Direct  cerebellar  and  Gowers'  tracts  are  invariably  involved. 
The  majority  of  observers  believe  that  the  crossed  pyramidal  bundle  is 
also  diseased,  but  considerably  less  than  the  posterior  columns,  and  the 
degeneration  commences  later.  The  affection  is  therefore  essentially  a 
combined  sclerosis. 

As  to  the  gray  matter,  the  cells  of  the  posterior  cornua  are  diminished 
in  number  and  size.  Clarke's  columns  present  the  most  changes:  the  cells 
are  atrophied  and  the  number  of  fibers  is  considerably  reduced.  The 
neuroglia  tissue  which  takes  the  place  of  the  normal  substance  is  prolifer- 
ated and  more  developed  in  the  dorsal  than  in  the  lateral  columns. 

The  meninges  are  sometimes  found  thickened  at  the  level  of  the 
posterior  columns.     The  dorsal  roots  are  also  in  a  state  of  sclerosis. 


262  Friedreich's  ataxia 

Usually  the  changes  just  described  are  confined  to  the  cord,  but  in  a 
few  cases  the  medulla  and  cerebellum  were  found  also  altered.  The 
involvement  of  the  latter  organ  led  Marie  to  describe  a  separate  type 
under  the  name  of  heredo-cerebellar  ataxia  (see  the  latter).  The  periph- 
eral nerves  have  also  been  found  atrophic  or  degenerated. 

Etiology. — The  fundamental  characteristics  of  Friedreich's  disease 
from  the  etiological  standpoint  is  that  it  occurs  in  several  members  of  the 
same  family.  The  symptoms  begin  to  appear  before  the  age  of  fourteen. 
Edinger  has  proposed  the  following  explanation  (Ersatz-Theorie).  When 
portions  of  the  nervous  system  are  not  developed  or  not  resistant,  over- 
work may  lead  to  their  degeneration.  Thus  the  beginning  of  the  symp- 
toms at  age  of  puberty  in  Friedreich's  disease  finds  its  reason. 

Cerebral  diseases  have  been  observed  in  relatives.  Syphilis,  alcohol, 
do  not  play  any  special  role.  Males  are  somewhat  more  frequently 
affected  than  females. 

Symptoms.  Motor. — The  first  symptom  to  appear  is  disturbance  of 
gait.  Slight  in  the  beginning,  it  gradually  becomes  more  and  more  marked. 
The  patient  walks  with  his  legs  widely  separated,  and  after  he  raises  them 
he  drops  them  heavily  on  the  ground.  At  the  same  time  the  entire 
body  oscillates  for  fear  of  falling.  This  ataxia  is  net  tabetic,  but  of  cere- 
bellar type.  The  station  is  also  ataxic.  While  Romberg's  sign  is  not 
present,  nevertheless  the  patient's  body  oscillates  to  and  fro,  his  feet  change 
position  very  often,  when  he  is  told  to  stand  still.  The  upper  extremities 
are  also  affected,  but  here  the  ataxia  appears  later,  long  after  the  legs 
become  affected.  Choreiform  movements  are  frequent:  the  gesticulations 
are  present  not  only  in  the  arms,  but  also  in  the  head  and  trunk.  As  the 
patient  sits  unsupported,  nodding  movements  of  the  head  and  swaying 
of  the  trunk  are  seen.  Athetoid  movements  may  also  occur.  The  limbs 
are  very  weak  and  as  a  rule  flaccid  but  there  are  no  paralytic  symptoms. 
Intention  tremor  is  quite  frequent.  The  reflexes  are  changed:  the  knee- 
jerks  are  usually  abolished,  but  Babinski's  sign  is  present.  In  some 
cases  the  knee-jerk  is  preserved  until  late  in  the  course  of  the  disease. 
The  abdominal  reflex  gradually  disappears.  The  tendon  reflexes  of  the 
arms  disappear  later  than  those  of  the  legs. 

Sensory. — General  sensations  are  usually  not  affected,  except  per- 
haps in  the  last  stage,  when  anaesthesia  appears.  Egger  observed  in  his 
cases  a  diminution  or  loss  of  vibratory  sense  of  tuning-fork.  The  special 
senses  are  not  involved.  The  eyes,  however,  present  one  special  symp- 
tom which  is  very  frequent  in  Friedreich's  disease,  viz.  nystagmus.  It 
consists  of  brief  and   repeated  movements   of  the  eye-globes  when  the 


FRIEDREICH  S    ATAXIA 


263 


patient    is  told  to  turn  his    eyes  to  the  right  and  to  the  left,  without 
moving  his  head.     The  ocular  movements  are  intact. 

Cerebral. — Vertigo  is  frequent.  Intelligence  is  normal,  except  to- 
wards the  end,  when  dullness  and  childishness  make  their  appearance. 
The  speech  is  almost  always  affected:  it  is  slow,  the  articulation  is  diffi- 
cult, irregular;  some  word.;  are  pronounced  abruptly,  some  with  delibe- 
ration. During  conversation  the  muscles  of  the  face  are  affected  with 
irregular  contractions  the  movements  of  the  face  are  ataxic. 


Fig.  86. — Characteristic  Feet  in  Friedreich's  Ataxia.     {Bouchard  and  Brissaud.) 


Trophic. — There  is  a  special  deformity  of  the  foot  which  is  character- 
istic of  the  disease.  It  is  of  the  equinus  type:  the  foot  is  short,  as  if  it 
was  pressed  antero-posteriorly;  the  plantar  surface  is  concave,  while  the 
dorsal  is  prominent;  the  toes  are  "claw-like"  because  of  forced  extension. 
This  condition  is  usually  bilateral.  In  a  few  cases  the  same  deformity 
was  observed  in  the  hands,  which  also  assume  the  claw-like  position 
(main  en  griff e) .  Scoliosis,  or  more  frequently  kypho-scoliosis,  is  observed 
in  an  advanced  stage;  it  is  particularly  marked  in  the  dorsal  region.  The 
sphincters  are  as  a  rule  intact. 

Course  and  Prognosis. — The  disease  is  essentially  progressive,  but 
slow  in  the  development.  The  earliest  symptoms  are :  ataxia  and  hyper- 
extension  of  the  great  toe.  The  upper  extremities  become  involved  after 
the  lower.  The  eye  symptom  and  the  peculiar  speech  appear  still  later. 
Within  a  period  of  five  years  the  clinical  picture  is  complete.  The 
patient  is  then  bedridden  and  loses  all  power  in  his  limbs.  Remissions 
have  been  observed,  but  any  intercurrent  acute  disease  hastens  the  course 
of  the  malady.  It  may  last  an  indefinite  number  of  years,  but  the  outlook 
is  invariably  grave.     Death  usually  occurs  from  some  intercurrent  disease. 

Diagnosis.— With    tabes    Friedreich's  disease    may    be   confounded 


264  INFANTILE    SPINAL  PARALYSIS 

because  of  the  ataxia  and  the  loss  of  knee-jerks.  In  the  latter  affection 
there  is  no  true  tabetic  incoordination  (see  Tabes),  but  a  cerebellar 
titubation.  Moreover  the  presence  of  choreiform  movements,  nystagmus, 
disturbance  of  speech,  the  absence  of  sensory  disturbances,  of  sphincter 
involvement,  of  pupillary  changes,  will  enable  one  to  make  the  diagnosis  of 
Friedrich's  disease. 

In  Multiple  Sclerosis  there  are  nystagmus  and  intention  tremor, 
but  in  Friedreich's  disease  the  nystagmiform  movements  of  the  eye-globes 
are  only  in  transverse  direction,  while  in  multiple  sclerosis  they  are  in  all 
directions.  The  tremor  in  Friedreich's  disease  is  considerably  less  marked 
than  in  multiple  sclerosis.  The  knee-jerks  in  the  latter  disease  are  usually 
increased,  in  the  former  abolished. 

Treatment. — It  is  purely  symptomatic.  There  are  no  remedies  for 
arresting  the  course  of  the  disease,  which  is  inevitably  progressive. 
Massage  and  systematic  exercises  of  the  limbs  (motor  reeducation)  maybe 
of  benefit  (see  Treatment  of  Tabes). 

V.  Infantile  Spinal  Paralysis  (Acute  Anterior  Poliomyelitis) 

Pathology. — The  lesion  consists  chiefly  of  an  acute  inflammation  of 
the  anterior  cornua  of  the  spinal  cord.  In  recent  cases  the  microscope 
shows  dilatation  of  the  blood  vessels,  leucocytic  infiltration  of  their  walls 
(arteritis);  the  ganglionic  cells  surrounded  by  this  inflammatory  tissue 
undergo  degeneration  and  atrophy:  their  protoplasma  (Nissl's  bodies) 
becomes  disintegrated,  the  nucleus  and  nucleolus  fall  out  and  the  pro- 
longations (dendrites  and  axis  cylinders),  whose  existence  depends  upon 
the  integrity  of  the  cells,  undergo  atrophy.  The  morbid  process  therefore 
consists  of  inflammatory  softening.  The  latter  may  be  found  in  small 
foci  or  occupy  a  large  portion  of  the  cornua.  The  destroyed  tissue  is 
gradually  substituted  by  proliferated  neuroglia,  so  that  when  the  cord 
is  examined,  several  years  after  the  acute  onset,  one  can  see  cicatricial 
tissue  in  the  substance  of  the  anterior  cornua,  and  in  the  midst  of  it  de- 
formed, dilated  and  thickened  blood  vessels.  The  entire  cornu  becomes 
diminished  in  size,  and  if  the  other  is  intact,  the  contrast  between  the 
two  is  striking.  The  anterior  roots,  which  are  the  prolongation  of  the 
axis-cylinders  emanating  from  the  cells,  undergo  atrophy.  The  motor 
fibers  of  the  body  which  are  the  continuation  of  these  roots  and  the 
muscles  supplied  by  the  nerves  of  the  anterior  roots  also  suffer  in  their 
nutrition :  the  muscular  fibers  undergo  granular  degeneration  and  are 
replaced  by  fibrous  or  adipose  tissues.  The  bones  of  the  affected  limbs 
participate  in  the  general  and  progressive  atrophy,  so  that  an  entire 


INFANTILE    SPINAL   PARALYSIS  265 

limb  may  be  arrested  in  its  development  and  remain  very  small,  if  the 
disease  occurred  in  infancy  and  was  neglected. 

Etiology  and  Pathogenesis. — All  the  evidences  are  in  favor  of  an 
infectious  cause  of  the  disease.     The  onset  of  the  disease  and  the  occasional 
epidemics  occurring  in  certain  localities  leave  no  doubt  as  to  its  infectious 
nature.     On  two  occasions  within  a  period  of  two  years  I  observed  a 
series  of  eight  cases  and  of  twelve  cases  in  one  section  of  this  city.     Ac- 
cording to  Marie,  an  embolus  of  infectious  nature  reaches  the  anterioi 
cornua  through  a  branch  of  the  anterior  spinal  artery.     The  most  recent 
experimental  investigations  of    Levaditi,  Landsteiner,  Popper,  Flexner, 
Lewis   and   others   have   established   the   fact   that 
the  upper   respiratory   passages   and  the  intestinal 
tract  are  the  usual  seat  of  the  virus.     Expectora- 
tion  and    the  stools   of    an  infected  individual  are  * 
the   sources   of    contamination    (Kling,    Weinstedt 
and  Pettersson).     Flexner,  Clark  and  Frazer    have 
demonstrated    {Jour.  Amer.  Med.  Assn.,  1913)  the                  ^ 
occurrence    of    the  virus    in   the   naso-pharynx   of 
healthy  persons  who  have  been  in  close  contact  with 
an  acute  case  of  poliomyelitis.     Contagion  by  dust, 

clothing     and    shoes    has    also    been    established.        ^     „       ~ 

0  _  Fig.  87. — Degenera- 

Insects,  such  as  flies,  have  been  shown  to  carry  TIOn  of  a  Cell  of  the 
the  virus  and  transmit  it.  The  original  idea  of  Anterior  Corntt  in  a 
Wickman   as   to   the  fact   that  the  disease  is  con-     Case   of  Acute  An" 

TERIOR  POLIOHYELITIS. 

tagious  is  now  completely  confirmed.  The  exis-  {original.) 
tence  of  epidemic  foci  may  pass  unobserved  in  large 
cities.  The  season  of  the  year  appears  to  be  an  important  predis- 
posing cause  of  poliomyelitis.  Summer  and  early  autumn  are  the 
most  favorite  seasons.  It  seems  that  a  certain  immunity  is  acquired  by  a 
country  in  which  an  epidemic  attack  once  occurred.  Thus  in  Sweden 
the  provinces  affected  severely  in  1905  were  apparently  free  from  the  dis- 
ease in  191 1.  The  immunity  produced  by  an  abortive  attack  will  prob- 
ably explain  the  resistance  of  older  children  and  adults. 

As  to  the  nature  of  the  virus,  it  is  as  yet  impossible  to  determine  it,  as 
the  microorganism  has  not  yet  been  discovered.  It  has  been  shown  by 
Flexner,  Lewis  and  Levaditi  that  the  unknown  microbe  belongs  to  the 
class  of  very  small  microorganisms  as  it  is  able  to  pass  through  the 
filter,  a  fact  which  is  not  observed  with  the  majority  of  well  defined 
microorganisms.  Finally  Rosenau  has  recently  demonstrated  that  the 
barn-fly  (stomoxys  calcitrans)  is  a  possible  disseminator  of  the  poliomy- 
elitic   infection.      Through    this    agency  he    succeeded   in    transmitting 


266  INFANTILE    SPINAL    PARALYSIS 

poliomyelitis  from  monkey  to  monkey  (/.  Am.  Med.  Ass.,  191 2).  Ander- 
son and  Frost  were  able  to  confirm  it;  they  also  succeeded  in  transmitting 
the  infection  to  a  fresh  monkey  with  the  emulsion  of  the  cord  of  an  animal 
infected  by  the  flies. 

Infantile  paralysis  is  a  disease  of  early  childhood.  It  should  be 
borne  in  mind  that  it  may  also  occur  in  adult  life,  but  this  is  rare.  The 
boys  are  more  frepuently  affected  than  girls.  Among  predisposing 
etiological  factors  trauma  may  be  mentioned.  I  have  seen  a  number 
of  cases  in  which  the  onset  dates  from  a  fall.  Starr  mentions  cases  of 
poliomyelitis  which  developed  immediately  after  exposure  to  cold,  such 
as  swimming  in  very  cold  water. 

Symptoms. — The  onset  is  always  sudden  and  resembles  that  of  an 
acute  infectious  disease.  The  incubation  period  usually  lasts  from  1  or 
2  days  to  10  days.  Fever,  general  malaise,  aonrexia,  vomiting,  diarrhoea 
or  else  coryza,  angina,  bronchitis  and  sometimes  convulsions  are  the 
usual  initial  symptoms.  In  some  cases  only  one  or  two  of  them  are 
present  and  in  others  they  are  so  mild  that  they  may  be  overlooked. 
In  still  others  none  of  the  premonitory  symptoms  is  observed.  The  child 
suddenly  becomes  paralyzed. 

Some  cases  begin  with  symptoms  of  meningitis  :  rigidity  of  the  neck 
and  trunk,  somnolence,  agitation,  headache,  convulsive  movements  of 
the  eyes,  tenderness  of  the  spine,  general  hyperesthesia,  articular  pain. 
Exanthemata  or  erythemata  are  not  infrequently  seen.  Children  fre- 
quently complain  of  pain  in  the  back  or  in  the  affected  limbs.  In  a  day 
or  two  and  sometimes  later  the  principal  symptom,  viz.  paralysis,  makes 
its  appearance,  and  it  progresses  so  rapidly  that  it  reaches  its  climax 
in  twenty-four  hours.  It  affects  one  limb,  two  symmetrical  limbs  (usually 
the  lower  extremities)  or  an  arm  and  leg  on  the  same  side,  or  one  arm; 
it  may  also  involve  an  arm  on  one  side  and  a  leg  on  the  other.  In  some 
cases  all  the  four  extremities  may  become  paralyzed.  In  a  few  days  the 
paralysis  retrocedes:  motility  returns  in  certain  groups  of  muscles,  so 
that  at  the  end  of  a  few  weeks  the  paralysis  becomes  fixed  in  one  or  two 
extremities.  When  the  lower  limbs  are  paralyzed,  there  may  be:  a  thigh 
type  and  a  leg  type.  In  the  former  the  muscles  on  the  inner  side  of  the 
thigh  escape;  the  iliacus,  glutei  and  the  antero-external  muscles  of  the 
thigh  are  affected.  In  the  leg  type  the  peronei  and  anterior  tibial  are 
mostly  paralyzed;  the  posterior  tibial  less  frequently.  When  the  upper 
limbs  are  involved,  there  may  be  also  two  types:  upper  and  lower  arm 
types.  In  the  first  the  scapular  muscles,  deltoid,  biceps  and  supinator 
longus  are  paralyzed.  In  the  second  the  muscles  below  the  elbow  except 
the  supinator  longus  are  affected.     Finally  an  entire  limb  may  be  involved. 


INFANTILE    SPINAL    PARALYSIS 


267 


The  most  charcteristic  feature  of  the  paralysis  is  the  flaccidity. 
The  tendon  reflexes  of  the  affected  limbs  are  either  totally  abolished  or 
greatly  diminished.  This  condition  is  the  general  rule.  The  following 
varieties  have  been  observed:  (1)  exaggeration  of  both  knee-jerks; 
(2)  loss  of  one  knee-jerk,  and  exaggeration  of  the  other;  (3)  a  transient 
exaggeration  immediately  preceding  their  abolition. 

The  paralyzed  muscles  soon  begin  to  degenerate  and  atrophy  and 
their  contractility  gradually  decreases.  Their  electrical  irritability  suffers 
a  radical  change:  under  faradism  there 
is  at  first  a  diminished  response  and 
later  complete  loss  of  response.  Under 
galvanism  there  is  not  only  a  quantita- 
tive, but  also  a  qualitative  alteration: 
reaction  of  degeneration  appears  quite 
early.  Later  all  contractility  to  elec- 
trical stimulation  is  lost. 

Parallel  with  the  increasing  atrophy, 
the  growth  of  the  muscles  is  arrested, 
contractures  are  formed  because  of  the 
predominance  of  the  antagonistic  mus- 
cles, and  all  these  factors  lead  to  de- 
formities. Among  the  latter  the  most 
common  is  pes  equinovarus.  Scoliosis, 
lordosis,  are  also  met  with.  Finally  the 
hands  may  also  occasionally  be  affected: 
a  claw-like  hand  is  the  most  usual  de- 
formity. Deformities  of  joints  is  a  fre- 
quent    consequence     of     poliomyelitis: 

paralysis     of     the    muscles    surrounding       Fia  88.— Infantile  Spinal  Para- 
....  .  lysis,    Showing   Atrophy   of   right 

the  joints  leads  to  a  relaxation  of   the  LoWER  ExTEEMITY)  AND  scoliosis. 

latter,  so   that   the  heads  of  the  bones 

fall    from    their    sockets.     In    a    certain    number    of   cases    the    bony 

tissue  of  the  limb  may  be  arrested  in  its  development  and  then  one  can 

see  an  adult  with  one  or  two  extremely  thin  limbs.     The  skin  covering 

such  a  limb  is  very  thin,  ulcerates  after  the  slightest  erosion,  is  always 

cold,    cyanosed.     The   sensations    and    the   sphincters    are   intact.     In 

some  cases  I  observed  a  marked  tenderness  in  the  paralyzed  limbs  (Figs. 

88,  89,  90). 

Course,  Termination,  Prognosis. — The  clinical  picture  just  described 

may  vary  considerably.     Thus  the  paralysis  may  be  only  transitory  and 

last  only  a  few  days  or  else  two  to  three  weeks.     In  such  cases  the  atrophy 


268 


INFANTILE    SPINAL   PARALYSIS 


will  never  develop.  In  other  cases  the  disease  spreads  and  involves 
the  nuclei  of  the  medulla,  and  if  the  ninth  and  tenth  nerves  are  involved, 
the  patient  dies  from  bulbar  symptoms. 

The  typical  cases  when  properly  and  early  enough  treated  may  regain 
considerable  of  the  lost  power  in  the  limbs,  but  they  never  recover  com- 
pletely. The  majority  of  cases  remain  wdth  some  infirmity:  either  the 
pes  equinus  persists  or  the  atrophy  with  some  impairment  of  locomotion 
becomes  permanent.  In  some  cases,  many  years  after  the  first  attack, 
the  patient  is  taken  with  another  in  some  other  limb,  which  then  runs  the 


Fig.  89. — Infantile  Spinal  Paralysis 
Showing  Deformities  of  the  Lower 
Limbs. 


Fig.  90. — Atrophy  of  Right 
Thigh.  Sequela  of  Acute 
Anterior  Poliomyelitis  in 
Childhood. 


same  course  as  the  old  spinal  palsy.  In  a  case  that  came  under  my  obser- 
vation (Amer.  Med.,  1903)  symptoms  of  amyotrophic  lateral  sclerosis  began 
to  develop  a.  propos  of  an  injury  years  after  the  infantile  spinal  paralysis. 
Generally  speaking,  the  prognosis  depends  a  great  deal  upon  the 
electrical  reactions  of  the  muscles.  Reaction  of  degeneration  is  usually 
an  unfavorable  sign  for  recovery  of  the  paralyzed  muscles.  Loss  of 
faradic  contractility  is  not  always  an  indication  that  the  muscular  con- 
tractility will  not  return.     I  have  seen  cases  of  return  of  this  form  of 


INFANTILE    SPINAL    PARALYSIS  269 

electrical  reaction  long  after  the  onset  of  poliomyelitis.  As  to  life,  the 
outlook  is  good  except  when  the  medulla  is  involved. 

Diagnosis. — The  sudden  onset  with  its  characteristic  prodromal 
symptoms,  the  flaccid  palsy,  the  loss  of  reflexes,  the  atrophy,  are  all 
typical  enough  for  recognition  of  the  disease.  Nevertheless  there  is  a 
certain  group  of  affections  with  which  acute  poliomyelitis  may  be  con- 
founded. There  is  a  form  of  poliomyelitis  in  which  the  most  conspicuous 
symptom  is  pain  along  the  spine  and  in  the  limbs.  The  pain  is  consid- 
ered as  being  due  to  spinal  meningitis.  In  some  cases  the  onset  is  so 
tumultuous  that  epidemic  cerebro-spinal  meningitis  is  thought  of.  Pain 
in  the  head,  rigidity  of  the  neck,  pain  along  the  spine,  Kernig's  sign  and 
even  disturbance  of  consciousness  may  be  present.  The  differential 
diagnosis  will  be  as  follows:  Meningeal  symptoms  of  poliomyelitis 
disappear  rapidly  and  they  are  distinctly  spinal.  In  cerebro-spinal 
meningitis  they  are  both  cerebral  and  spinal.  In  the  first  the  paralysis 
is  paraplegic  monoplegic  or  hemiplegic  and  flaccid  in  type.  In  the  second 
the  paralysis  is  spastic  and  cranial  nerves  are  frequently  involved.  The 
reflexes  are  diminished  or  lost  in  the  first,  but  increased  in  the  second. 
The  toe  phenomenon  is  absent  in  the  first,  present  in  the  second.  Lympho- 
cytosis in  the  cerebro-spinal  fluid  is  characteristic  of  the  first,  leucocytosis 
of  the  second.  In  the  first  the  fluid  is  clear,  in  the  second  cloudy.  In 
the  first  there  are  very  few  polymorphonuclear  cells,  in  the  second — 
abundant.  No  microorganism  is  found  in  the  cerebro-spinal  fluid  of  poli- 
omyelitis, meningococci  are  found  in  that  of  cerebro-spinal  meningitis. 

In  serous  spinal  meningitis  in  which  pain  and  paralysis  also  lympho- 
cytosis are  present,  the  type  of  paralysis  and  the  condition  of  reflexes  are 
not  the  same  as  in  poliomyelitis. 

In  Multiple  Neuritis  are  present:  pain  in  the  limbs,  paralysis  and  altered 
reflexes  like  in  poliomyelitis.  The  points  of  difference  are:  in  the  second 
there  are  general  manifestations  indicative  of  an  acute  infection;  they  are 
absent  in  the  first.  In  the  second  paralysis  extends  further  than  in  the 
extremities,  the  muscles  of  the  trunk  are  involved.  In  the  first  the 
trunk  is  intact.  In  the  second  the  palsy  is  irregular  and  more  marked 
in  the  muscles  of  the  hip  and  thigh.  In  the  first  the  paralysis  is  regular 
and  more  marked  in  the  most  peripheral  muscles  of  the  limbs.  Atrophy 
sets  in  much  earlier  in  multiple  neuritis  than  in  poliomyelitis.  Objective 
sensations  are  rarely  disturbed  in  poliomyelitis;  in  polyneuritis  hyper- 
esthesia is  common  and  pain,  burning,  etc.,  are  frequently  complained  of 
in  the  fingers  and  toes.  Polyneuritis  is  frequently  cured  without  leaving 
any  trace,  but  this  is  not  the  case  with  poliomyelitis,  in  which  deformities 
of  the  limbs  are  the  rule. 


270  INFANTILE    SPINAL    PARALYSIS 

Acute  Transverse  Myelitis  is  exceptional  in  children.  Besides, 
involvement  of  the  sphincters  is  common.  In  poliomyelitis  the  sphincters 
are  not  involved. 

Birth  or  Obstetrical  Palsy  follows  a  difficult  labor  or  instrumental 
delivery;  there  are  always  sensory  disturbances.  The  atrophy  and 
objective  sensory  disturbances  followed  a  radicular  distribution. 

Cerebral  Infantile  Hemiplegia  will  be  recognized  by  the  spastic  paraly- 
sis and  the  abnormal  reflexes  usually  found  in  hemiplegia  (see  this  chapter). 

In  Hysterical  Paralysis  the  reflexes  are  preserved  and  the  electrical 
reactions  are  normal. 

The  foregoing  study  permits  the  following  grouping  of  varieties  of 
acute  poliomyelitis: 

(1)  The  Spinal  Type. — It  is  the  most  frequently  observed. 

(2)  The  meningeal  type,  in  which  the  onset  presents  meningeal  symp- 
toms (see  above) .  Generally  in  a  few  days  they  clear  up  and  the  picture 
of  poliomyelitis  becomes  evident. 

(3)  The  polyneuritic  type,  in  which  there  is  hyperaethesia  and  pain  in  the 
affected  limbs  (see  above). 

(4)  The  bulbar  type,  in  which  the  nuclei  in  the  medulla  become  involved 
in  the  course  of  poliomyelitis:  the  prognosis  here  is  grave. 

(5)  The  abortive  type,  in  which  the  symptoms  are  very  mild,  last  but 
a  short  time  and  totally  disappear. 

Treatment. — At  the  onset  of  the  disease  the  child  should  be  kept 
in  bed  and  every  effort  made  to  reduce  the  temperature  with  cold  - 
baths  or  spongings  and  coal-tar  products  (antipyrin,  phenacetin). 
Cauterization  and  revulsion  in  the  form  of  cupping  or  mild  counter- 
irritation  with  mustard  plasters  along  the  spine  may  be  of  benefit. 
Urotropin  taken  by  the  mouth  sets  free  formaldehyde,  and  the 
latter  is  then  found  in  the  cerebro-spinal  fluid.  It  acts  therefore  as  an 
antiseptic.  It  may  therefore  be  useful.  A  child  of  three  can  be  given  1-2 
gr.  two  or  three  times  a  day.  Pain  can  be  combated  by  sedatives 
such  as  bromides.  Purgatives  should  never  be  neglected.  The  naso- 
pharynx should  be  taken  special  care  of  (see  Etiology),  and  treated 
with  antiseptic  irrigations  or  applications.  Ergot  has  been  advised 
internally.  After  the  acute  stage  has  subsided,  an  effort  is  to  be  made  to 
improve  the  general  nutrition  by  a  substantial  diet  and  hydrotherapy. 
Iodides  can  then  be  administered;  cod-liver  oil,  arsenic,  hypophosphites, 
are  useful.  Electrical  treatment  and  massage  must  be  instituted  as 
promptly  as  possible.  It  is  advisable  at  first  to  apply  the  galvanic  cur- 
rent with  the  positive  pole  on  the  affected  muscles.  When  improvement 
is  noticeable,  the  faradic  current  will  be  more  useful.     Daily  applica- 


INFANTILE    SPINAL    PARALYSIS  2J I 

tions  of  electricity  for  ten  or  fifteen  minutes,  aided  by  massage  of  the 
limbs  and  kept  up  persistently  will  render  great  service. 

When  improvement  is  commencing  to  be  noticeable,  the  patient  is 
taught  to  exercise  the  affected  muscles  voluntarily.  Such  efforts  on  the 
patient's  part  are  of  great  benefit.  As  to  the  duration  of  the  electrical 
treatment  and  massage,  it  should  be  kept  up  indefinitely.  I  have  seen 
cases  which  began  to  show  signs  of  improvement  at  the  end  of  one  to 
two  and  even  three  years. 

To  guard  against  contractures  systematic  exercises  and  gymnastics 
are  advisable.  When  contractures  and  deformities  are  present,  orthopedic 
applicances  may  be  of  some  benefit.  The  use  of  mechanical  apparatuses 
should  not  begin  too  early,  as  the  weight  of  them  will  impose  too  much 
work  on  the  muscles  which  commence  to  improve  and  thus  overfatigue 
them.  I  have  seen  bad  results  from  too  early  application  of  braces.  On 
the  other  hand  a  properly  applied  apparatus  may  prevent  a  tendency  to 
deformity.  The  time  of  using  braces  must  be  individualized  in  each  case; 
no  set  rule  can  be  given  for  all  cases.  If  after  a  sufficiently  long  trial  the 
orthopedic  appliances  fail,  surgical  intervention  should  be  resorted  to. 
In  certain  cases  tenotomy  of  shortened  tendons  will  place  the  deformed 
limb  in  a  straight  position,  provided  there  is  enough  muscle  left  to  be 
relied  upon.  Recently  attempts  have  been  made  to  connect  muscles  which 
are  intact  with  the  atrophied  muscles.  A  healthy  tendon  or  muscle 
is  divided  and  sewed  to  the  atrophied  tendon  or  muscle,  respectively. 

I  have  seen  some  very  good  results  from  such  procedures.  Instead 
of  anastomosing  muscles,  healthy  nerves  have  been  divided  and  their 
central  ends  united  with  the  peripheral  ends  of  divided  nerves  in  the 
atrophied  muscles.     Some  good  results  have  been  reported. 

Recently  Schwab  and  Allison,  also  Stoffel,  devised  methods  to  com- 
bat contractures  and  deformities.     (See  pages  122  and  123.) 

Prophylaxy  of  Acute  Poliomyelitis.- — The  above  described  results 
obtained  from  experimental  investigations  (see  Etiology)  gives  us  a  clear 
idea  of  what  preventive  measures  should  be.  It  is  necessary  also  to  em- 
phasize the  fact  that  the  virus  may  resist  a  very  long  time.  For  example, 
the  virus  mixed  with  sterile  milk  or  water  kept  at  temperature  of  the  room 
maintains  its  pathogenic  activity  during  thirty-one  days  (Landsteiner  and 
Levaditi).  Salivary  and  nasal  secretions  are  carriers  of  poliomyelitic 
virus  and  are  chiefly  the  sources  of  contagion.  As  desiccation  does  not 
destroy  the  activity  of  the  virus,  contagion  may  take  place  through 
dried  up  secretions.  Rigorous  antiseptic  measures  applied  to  the  nose 
and  throat  in  cases  of  epidemics,  or  when  in  contact  with  individuals 
affected  with  the  disease,  are  urgent.     Isolation  is  equally  urgent. 


272  MYELITIS 

VI.  Chronic  Anterior  Poliomyelitis  and  Amyotrophic  Lateral  Sclerosis 

They  belong  to  the  systemic  diseases  of  the  spinal  cord  and  are  described 
in  the  chapter  on  Muscular  Atrophies. 

B.    NON-SYSTEMIC  DISEASES  OF  THE  SPINAL  CORD 

I.  Myelitis 

A.  Acute  Myelitis 

Acute  myelitis  is  an  acute  inflammation  of  the  spinal  cord  secondary 
to  an  infection  or  intoxication. 

Pathology. — The  lesion  is  essentially  diffuse.  It  is  irregular  in 
distribution.  When  several  foci  are  present,  the  disease  is  a  disseminate 
myelitis ;  when  one  focus  extends  upward  or  downward,  it  presents  a 
diffuse  myelitis.  When  the  entire  transverse  section  of  the  cord  is 
involved,  the  disease  is  a  transverse  myelitis.  According  to  the  seat  of 
the  lesion,  myelitis  may  be:  cervical,  dorsal,  lumbar. 

Irrespective  of  the  localization  of  the  focus,  the  pathological  anatomy 
in  all  varieties  of  myelitis  is  the  same.  The  cord  is  usually  congested, 
cedematous,  and  the  diseased  portions  are  soft.  The  softening  undergoes 
three  distinct  phases :  at  first  the  stage  of  red  softening,  later  that  of  white 
softening  and  still  later  the  stage  of  sclerosis. 

In  the  first  period  (red  softening)  the  blood  vessels  are  dilated  and 
their  walls  are  infiltrated  with  leucocytes.  The  cells  of  the  gray  matter 
undergo  disintegration,  and  the  axis-cylinders  are  swollen  and  varicose. 

In  the  second  period  (white  softening)  necrosis  takes  place:  the  entire 
cord  tissue  is  very  soft  and  appears  white;  the  cells  are  colorless  and  de- 
formed, without  nuclei  and  prolongations.  The  nerve  fibers  lose  their 
myelin  sheaths,  remain  naked  and  unsupported.  Ascending  and  descend- 
ing degeneration  in  the  white  columns  becomes  evident.  Instead  of  the 
normal  nerve  tissue  there  is  a  large  number  of  granular  cells. 

In  the  third  period  there  is  formation  of  cicatrices  at  the  expense  of 
the  neuroglia  tissue,  which  at  that  time  proliferates  in  abundance:  instead 
of  being  soft  the  cord  becomes  hard.  As  death  may  occur  during  any  one 
of  these  stages,  the  histological  picture  will  vary  according  to  when  the 
pathological  process  was  arrested  in  its  development. 

The  meninges  usually  participate  in  the  myelitic  process.  They 
are  thickened  (especially  the  pia-mater)  and  adhere  to  the  cord.  If 
the  cells  of  the  anterior  cornua  are  extensively  involved,  the  anterior 
roots  and  the  peripheral  nerves  undergo  alterations  and  the  muscles 
supplied  by  these  nerves  atrophy.     With  reference  to  the  pathology  of 


MYELITIS  273 

myelitis  it  is  interesting  to  say  a  few  words  of  Buzzard's  conception. 
He  believes  that  myelitis  is  the  result  of  a  thrombotic  softening  of  the 
cord  and  that  the  usual  causes  of  myelitis  produce  an  inflammatory 
process  in  the  vessel  wall  which  permits  the  formation  of  thrombi,  resulting 
in  softening  of  the  cord.  The  special  nature  of  distribution  of  the  blood 
vessels  in  the  spinal  cord  is  such  as  to  directly  favor  the  occurrence  of 
thrombosis,  especially  in  the  lumbar  region  where  myelitis  is  so  frequently 
met  with.  This  peculiarity  consists  of  a  great  length  of  the  small  arteries, 
especially  of  those  supplying  the  lower  portion  of  the  cord.  Acute  myeli- 
tis is  therefore,  according  to  Buzzard,  a  thrombotic  softening. 

Etiology. — Acute  myelitis  is  always  the  result  of  an  infection  or  intoxi- 
cation. Cold,  traumatism,  emotions  which  formerly  were  considered  as 
direct  causes,  are  in  reality  only  predisposing  factors.  In  favor  of  this 
view  speak  the  occurrence  of  myelitis  in  the  course  of  acute  and  chronic 
infectious  diseases,  also  experimental  investigations.  Influenza,  typhoid 
fever,  small-pox,  scarlet  fever,  pneumonia,  gonorrhoea,  whooping  cough, 
measles,  tuberculosis  and  syphilis  are  all  diseases  which  may  become 
complicated  by  myelitis.  A  neighboring  inflammatory  process  in  the 
meninges  or  vertebrae  may  extend  to  the  cord  and  develop  myelitis. 

During  puerperium  myelitis  has  been  observed.  In  all  these  various 
infections  of  a  general  or  local  nature,  either  the  microorganism  itself 
or  its  toxin,  and  perhaps  both,  are  the  immediate  causes  of  myelitis: 
they  penetrate  the  cord  through  the  blood  vessels.  Experimentally 
it  has  been  proven  beyond  any  doubt,  I  believe,  that  myelitis  can  be  pro- 
duced by  inoculations  of  various  microbes  and  of  their  toxins  (Roux, 
Yersin,  Grancher,  Manfreda,  etc.). 

Symptoms. — In  the  chapter  on  "Pathology"  various  forms  of  myelitis 
have  been  mentioned.     The  most  frequent  of  all  is 

Transverse  Myelitis. — The  usual  seat  of  this  form  is  the  dorso-lumbar 
region,  but  it  may  also  occur  in  the  cervical  and  dorsal  regions.  The  onset 
in  the  majority  of  cases  preceded  by  prodromal  symptoms.  They  are: 
pain  in  the  back,  paresthetic  disturbances  (tingling,  numbness,  etc.), 
retention  of  urine,  also  some  weakness  in  the  extremities.  The  pain  is 
not  confined  to  the  back,  but  it  also  radiates  toward  the  limbs.  The 
prodromal  period  usually  lasts  from  a  few  hours  to  several  days.  After 
myelitis  is  established,  the  following  symptoms  become  characteristic. 

Motor, — In  the  dorso-lumbar  form  the  legs  present  a  complete  loss  of 
power.  If  the  myelitis  is  cervical,  the  arms  are  paralyzed. .  The  paralysis 
is  flaccid  in  transverse  myelitis  of  the  dorso-lumbar  cord,  spastic  in  dorsal 
and  cervical  myelitis.  The  patellar  tendon  reflexes  are  abolished  in  the 
first,  increased  in  the  other  two  forms.     Babinski's  sign  and  paradoxical 


2  74  MYELITIS 

reflex  may  be  present  in  all  the  three  forms.  Curiously  enough  there  is 
an  antagonism  between  the  latter  two  reflexes.  At  the  beginning  Bab- 
inski's  phenomenon  is  quite  rare,  but  the  paradoxical  very  frequent. 
Later  on,  when  contractures  appear,  the  paradoxical  becomes  less  marked, 
but  Babinski's  reflex  more  pronounced.  Oppenheim's  reflex  may  some- 
times be  present.     Ankle-clonus  may  and  may  not  be  present. 

As  the  disease  gradually  advances  and  passes  into  a  chronic  state,  con- 
tractures develop.  In  the  cervical  form  of  myelitis  the  paralysis  may 
affect  not  only  the  arms,  but  also  muscles  of  the  neck,  the  intercostal  and 
abdominal  muscles,  the  lower  extremities,  and  the  diaphragm,  and  thus 
cause  difficult  breathing.  In  myelitis  of  the  eighth  cervical  segment 
there  is  also  paralysis  of  the  small  muscles  of  the  hands  and  of  the  flexors 
of  the  fingers. 

Sensory.- — They  are  constant.  The  patients  complain  of  violent  and 
continuous  pain  in  the  back,  which  in  the  cervical  form  radiates  to  the 
arms  and  in  lumbar  form  to  the  legs.  The  tabetic  girdle  pain  is  present 
here.  Sensations  of  cold  or  heat,  tingling,  burning,  etc.,  are  also  very  fre- 
quent. Objective  sensory  disturbances  are  quite  marked.  The  sense 
of  touch,  pain  and  temperature  in  the  upper  and  lower  extremities  and  on 
the  body  (according  to  the  seat  of  the  lesion)  is  greatly  diminished  and 
sometimes  completely  lost  in  the  areas  innervated  by  the  diseased  seg- 
ments and  below  these  segments.  In  some  cases  there  is  a  dissociation 
of  the  three  forms  of  sensations  (see  Syringomyelia). 

Trophic  disturbances  are  usual.  The  affected  extremities  are  cyanosed 
and  cedematous.  When  a  portion  of  the  body  undergoes  pressure,  ulcer- 
ation soon  develops.  This  is  particularly  frequent  in  the  sacral  region, 
but  it  occurs  also  on  the  gluteal  areas  and  at  the  level  of  the  trochanters. 
The  decubitus  is  only  present  in  the  dorsal  and  dorso-lumbar  varieties 
of  transverse  myelitis.  The  ulceration  is  usually  superficial,  but  it  may 
be  so  deep  as  to  expose  the  bone.  In  a  case  that  recently  came  under  my 
observation,  there  was  at  first  a  cervical  myelitis,  but  later  the  lesion  spread 
in  a  descending  manner.  The  bedsores  were  so  marked  that  over  the 
entire  lumbar  region  the  cutaneous  and  muscular  tissues  were  totally 
destroyed  and  the  largest  part  of  the  lumbar  vertebrae  and  of  the  sacrum 
were  exposed.  In  the  same  patient  the  prepuce  was  totally  gangrenous 
and  a  portion  of  the  dorsum  of  the  penis  was  ulcerated.  Muscular  atrophy 
of  the  lower  extremities  is  rare,  but  frequent  in  the  upper  limbs  in  myelitis 
of  the  lower  cervical  cord,  absent  when  the  upper  cervical  segment  of  the 
cord  is  involved.  If  atrophy  occurs,  reactions  of  degeneration  (CaCC  > 
AnCC)  are  present.     In  myelitis  with  involvement  of  the  eighth  cervical 


MYELITIS  275 

segment  there  will  be,  besides  the  above  mentioned  paralysis  and  atrophy, 
also  small  pupils  and  narrow  palpebral  fissure. 

Sphincters  are  always  involved  in  the  dorsal  and  dorso-lumbar  mye- 
litis. At  the  beginning  there  is  retention.  Later  incontinence  develops. 
Both  sphincters  are  almost  equally  involved.  Cystitis  is  a  frequent 
complication.  Impotence  frequently  exists  in  myelitis  of  any  segment 
of  the  cord. 

The  clinical  pictures  of  myelitis  at  various  levels  of  the  cord  as  just 
given  are  not  always  clear-cut.  They  may  vary  from  one  case  to  an- 
other. It  depends  upon  the  extent  of  the  transverse  section.  If,  for 
example,  in  the  cervical  form  the  lesion  is  sufficient  to  cut  off  all  impulses 
passing  through  the  diseased  segment,  the  paralysis  of  the  body  below  the 
lesion  will  be  flaccid,  all  reflexes  and  sensations  are  lost.  So  it  is  with 
any  other  segment  of  the  cord.  If  the  lesion  affects  only  one  side  of  the 
cord,  Brown-Sequard's  form  of  paralysis  will  be  present. 

Syphilitic  myelitis,  which  is  the  most  frequent  of  all  form,  presents 
certain  special  features.  It  will  be  discussed  in  detail  in  the  chapter 
on  Syphilis  of  the  Nervous  System. 

Tubercular  myelitis  may  occur:  (1)  as  a  complication  of  cerebro-spinal 
tubercular  meningitis  (tubercular  meningo-myelitis) ,  (2)  as  a  complica- 
tion in  Pott's  disease  (either  as  a  compression  myelitis  or  a  secondary 
myelitis  following  a  caseous  pachymeningitis) ;  (3)  it  may  also  occur 
in  the  course  of  Pott's  disease  as  primary  parenchymatous  myelitic  foci 
which  develop  without  meningitis,  without  vascular  and  interstitial  lesions, 
without  caseation  and  very  probably  due  to  tuberculine  circulating  in 
the  cord.  This  form  of  myelitis  is  analogous  to  lesions  found  in  the  liver 
and  kidneys  of  tubercular  individuals.  Philippe  and  Cestan  have  re- 
ported such  a  form  of  myelitis  (Rev.  Neurol.,  1899). 

Course,  Termination,  Prognosis. — While  in  the  majority  of  cases 
the  complete  picture  of  acute  myelitis  develops  within  a  period  of  from 
a  few  days  to  a  few  weeks,  there  are  nevertheless  cases  with  a  sudden 
onset  and  rapid  death.  Usually  the  disease  is  progressive  and  becomes 
chronic.  Great  improvement  and  even  complete  recovery  are  possible, 
especially  in  cases  that  follow  an  acute  infectious  disease.  In  such  cases 
the  improvement  in  sensations  precedes  that  in  the  motor  power.  The 
paraplegia  is  usually  spastic.  The  improvement  may  be  maintained 
for  many  years  and  the  patient  may  resume  his  usual  occupation  and  be 
only  slightly  disturbed  by  the  sphincters.  The  greatest  amount  of  dis- 
turbance occurs  in  myelitis  of  the  lowest  segment  of  the  cord.  Here  the 
atrophy  of  the  muscles  and  the  condition  of  the  sphincters  are  exceed- 
ingly obstinate.     In  some  cases  the  disease  acquires  an  ascending  or  de- 


276  MYELITIS 

scending  course.  In  the  first  case  it  may  reach  the  medulla  and  then  lif  e 
is  greatly  threatened.  In  other  cases  the  decubitus  and  cystitis,  which 
may  become  purulent,  are  capable  of  killing  the  patient.  Finally  inter- 
current diseases  are  a  source  of  great  danger  to  myelitic  patients.  Re- 
missions in  the  course  of  myelitis  are  possible.  The  patient  apparently 
improves  to  a  great  extent,  and  under  the  influence  of  an  insignificant  cause 
may  suffer  a  relapse.     The  outlook  in  acute  myelitis  is  always  doubtful. 

Diagnosis.— Multiple  neuritis  is  sometimes  confounded  with  myelitis. 
Tenderness  of  the  nerve  trunks,  the  predominance  of  the  sensory  disturb- 
ances, integrity  of  the  sphincters,  irregular  involvement  of  the  motor  ap- 
paratus and  especial  involvement  of  extensor  groups  of  muscles — all 
these  symptoms  are  characteristic  of  multiple  neuritis.  Besides,  in 
myelitis  the  objective  sensory  disorder  is  of  segmental  type,  and  more  pro- 
found. In  neuritis  the  limits  of  the  sensory  disturbances  are  not  well 
defined,  the  latter  are  present  only  at  the  periphery,  and,  what  is  im- 
portant, the  tenderness  of  the  nerve  trunks  is  associated  with  anaesthesia 
or  analgesia  of  the  skin. 

Hysterical  paraplegia  may  simulate  acute  myelitis.  In  the  former 
there  is  usually  a  history  of  a  shock  or  of  a  great  emotion,  of  a  traumatism. 
The  onset  is  sudden.  The  reflexes  and  the  sphincters  are  intact.  There 
are  no  trophic  disturbances.  Finally  the  existence  of  hysterical  stigmata 
will  aid  in  making  the  diagnosis. 

Hematomyelia  presents  a  very  sudden  onset  besides  the  history  of 
trauma. 

Spinal  compression  presents  usually  a  gradually  oncoming  paralysis, 
but  when  the  paralysis  is  sudden  or  rapid  like  in  transverse  myelitis,  it 
is  usually  caused  either  by  caries  or  malignant  disease  of  the  vertebrae. 
Besides,  the  pain  in  compression  myelitis  is  unusually  intense  and 
agonizing. 

Treatment. — In  the  first  stage  of  the  disease  the  patient  should  be 
given  absolute  rest.  A  purgative  should  be  administered.  Pain  will  be 
controlled  by  salicylates  and  coal-tar  products.  In  case  of  retention  of 
urine  catheterization  is  necessary.  Local  applications  to  the  spine,  as 
vesicants  or  cauterization,  have  been  recommended,  but  in  view  of  the 
sensory  disturbances  which  are  constantly  present  in  myelitis,  and  the 
facility  with  which  ulcerations  are  formed,  it  is  advisable  to  avoid  local 
irritation  of  the  skin.  Inunctions  of  mercury  and  internal  administration 
of  iodids  should  be  instituted  as  soon  as  possible  not  only  in  syphilitic 
cases,  but  also  when  no  history  of  direct  infection  could  be  obtained. 
(For  the  mode  of  administration  and  dosage,  see  Tabes.) 

In  addition  to  the  drug  treatment  hygienic  measures  are  of  great  im- 


MYELITIS  277 

portance.  Special  and  prompt  care  must  be  taken  of  the  bladder  and  of 
the  decubitus,  as  complications  not  infrequently  come  from  these  two 
sources  and  death  may  ensue.  Purulent  cystis  is  often  the  result  of  infec- 
tion from  repeated  catheterization.  Decubitus  is  also  infectious  in  nature. 
It  is  therefore  essential  to  see  that  urine  and  feces  should  not  come  in  con- 
tact with  the  skin,  that  aseptic  and  antiseptic  precautions  be  taken  in  the 
strictest  possible  manner.  In  cases  of  retention  of  urine,  each  aseptic  cath- 
eterization must  be  followed  by  a  washing  out  of  the  bladder  with  some  mild 
antiseptic  solution,  as  boric  acid.  By  doing  so,  cystitis  with  all  its  grave 
consequences  may  be  avoided.  In  order  to  avoid  undue  pressure  of  the 
skin,  as  the  latter  is  usually  the  immediate  cause  of  decubitus,  the  patient's 
position  in  bed  should  be  frequently  changed  and  the  bed  cloth  kept 
smooth. 

After  the  acute  stage  has  subsided,  massage  becomes  indicated,  but  in 
this  latter  procedure  great  care  must  be  exercised  that  the  skin  is  not  in- 
jured. In  a  patient  under  my  observation  a  small  erosion  of  the  skin  of 
one  leg  caused  by  a  masseuse  became  ulcerated  and  during  three  and  a 
half  months  the  ulceration  remained  practically  unaffected  by  various 
medications.  Electricity  is  a  valuable  adjuvant  to  massage.  It  should 
be  avoided  in  cases  of  spasticity,  but  it  may  be  useful  in  flaccid  paralysis. 
Both  procedures  are  important  in  treatment  of  atrophy.  Galvanism  at 
first  and  faradism  later  or  both  alternately  may  be  employed. 

Hydrotherapy  in  the  form  of  sponging,  or  ablutions  followed  by  gentle 
rubbing,  is  a  good  adjuvant  in  treatment  of  myelitis. 

Spasticity  of  the  extremities  can  be  relieved  by  passive  movements 
and  warm  baths. 

Acute  Diffuse  Myelitis 

This  is  a  rare  disease.  Pathologically  it  consists  of  a  myelitic  focus 
having  an  ascending  (more  frequent)  or  descending  course.  It  is  known 
under  the  name  of  acute  ascending  or  Landry's  paralysis.  It  will  be 
described  later  together  with  multiple  neuritis. 

Acute  Disseminated  Myelitis 

This  affection  consists  pathologically  of  myelitic  foci  disseminated 
throughout  the  central  nervous  system.  Its  clinical  picture  is  that  of 
disseminated  sclerosis.     The  latter  will  be  described  later. 

B.  Chronic  Myelitis 

Chronic  myelitis  as  a  primary  affection  occurs  in  traumatism,  in  com- 
pression of  the  cord,  in  infections  of  long  standing  (syphilis,  leprosy),  in 


278 


MYELITIS 


intoxications  of  long  standing  (diabetes,  pernicious  anemia,  ergotism). 
The  lesion  and  the  symptoms  are  slow  and  long  in  development.  They 
will  be  described  in  separate  chapters. 

As  a  secondary  affection  it  follows  acute  myelitis.  It  is  only  with  this 
form  that  we  will  be  concerned  here. 

Pathology.- — If  a  myelitic  cord  is  examined  years  after  the  acute 
onset,  it  will  be  found  shrunken  and  small  in  size.  A  transverse  cut  will 
show  that  the  cord  is  discolored  and  grayish  in  appearance.  Histologically 
it  presents  a  destruction  of  cells  and  disappearance  of  myelin  around  the 
nerve  fibers,  also  in  some  places  disappearance  of  axis-cylinders.  Instead 
of  the  normal  nervous  elements,  there  is  seen  a  large  amount  of  proliferated 
neuroglia  and  connective  tissue.  Secondary  ascending  and  descending 
degenerations  are  distinct.  The  meninges  are  frequently  found  thickened 
as  a  result  of  a  previous  participation  in  the  inflammatory  process.  The 
walls  of  the  blood  vessels  are  also  thickened. 


Fig.  91. — Chronic  Myelitis,  Showing  Degeneration  op  White  and  Gray  Substance. 

{Original.) 

Symptoms. — They  are  practically  the  same  as  in  acute  myelitis,  when 
the  latter  are  fully  developed,  viz.  spastic  paraplegia  with  the  usual 
abnormal  reflex  phenomena,  disturbances  of  the  sphincters,  sensory 
disturbances,  as  paresthesia,  hyperesthesia  or  anaesthesia.  What  char- 
acterizes chronic  myelitis  particularly  is  the  mild  degree  of  the  disturbances 
in  the  sphincters  and  in  the  sensory  sphere.  The  spasticity  on  the  con- 
trary is  much  pronounced.  Atrophy  of  the  affected  limbs  also  occurs  and 
the  electrical  contractility  is  usually  diminished.  Reactions  of  degenera- 
tion are  rare.     The  extent  of  the  loss  of  power  in  the  extremities  is  various. 


HEMATOMYELIA 


279 


In  some  cases  it  is  absolute,  in  others  only  partial  and  in  still  others 
extremely  slight.     Bed-sores  are  rare. 

Course,  Prognosis. — While  the  disease  is  usually  progressive,  in  a 
great  many  cases  it  presents  stationary  periods.  At  all  events  it  is  ex- 
tremely slow  in  development.  The  prognosis  is  unfavorable,  as  the 
destroyed  tissue  can  never  be  replaced  by  healthy  nervous  elements. 
Death  usually  occurs  from  some  intercurrent  disease  or  from  bulbar  in- 
volvement, when  the  myelitis  is  of  ascending  character  or  when  confined 
to  the  cervical  cord. 

Diagnosis. — The  history  of  the  onset  and  the  grouping  of  the  charac- 
teristic symptoms  are  usually  sufficient  for  making  the  diagnosis.  Some- 
times, however,  the  diagnosis  may  present  some  difficulty.  In  progressive 
muscular  atrophy,  for  example,  the  muscular  wasting  is  regular  and  typical 
in  distribution,  while  in  chronic  myelitis,  if  atrophy  occurs,  it  is  irregular. 
Moreover  in  the  latter  affection  there  are  sensory  disturbances  and  they 
are  absent  in  the  first.  Finally  the  state  of  the  sphincters  will  remove  the 
least  doubt,  as  they  are  intact  in  progressive  muscular  atrophy. 

Primary  Spastic  Paraplegia  will  be  recognized  by  the  absence  of 
sphincter  disorder,  of  sensory  disturbances  and  of  a  history  of  an  acute 
onset. 

A  tumor  of  the  spine  will  be  recognized  by  its  slow  development  and 
by  the  unusual  intensity  of  pain. 

Treatment. — -It  is  largely  the  same  as  that  of  the  advanced  stage  of 
acute  myelitis  (see  above). 


II.  Hematomyelia  (Hemorrhage  in  the  Spinal  Cord) 

Hemorrhage  in  the  cord  may  occur  in  the  course  of  acute  myelitis 
or  specific  myelitis,  tumors,  pernicious  anemia  and  in  cases  of  softening 
caused  by  an  embolus  or  thrombosis.  In  such  cases  the  hemorrhage 
is  a  secondary  condition,  merely  an  accident  in  the  course  of  those 
affections. 

Hemorrhage  in  the  cord  may  also  be  primary.  It  is  with  the  latter 
exclusively  that  we  will  be  concerned  here. 

Pathology. — The  hemorrhage  may  be  capillary  or  in  the  shape  of  one 
or  several  foci  of  more  or  less  large  size.  The  favorite  seat  for  primary 
hemorrhages  is  the  gray  matter,  because  of  the  great  vascularity  of  the 
latter.  They  are  particularly  marked  at  the  level  of  the  enlargements. 
They  may  extend  transversely  or  longitudinally;  they  may  also  occur  in 
the  central  canal.     The  cervical  region  is  the  most  frequent  seat  of  trau- 


280  HEMATOMYELIA 

matic  hematom\-elia;  the  dorsal  is  next  in  frequency.  On  section  the  cord 
appears  soft.  At  first  the  blood  destroys  the  cells  and  the  axis- cylinders 
undergo  secondary  degeneration.  Later  on,  when  the  clot  begins  to  disap- 
pear, the  neuroglia  tissue  around  it  proliferates.  Above  and  below  the 
hemorrhagic  foci  tracts  of  nerve  fibers  will  undergo  ascending  and  descend- 
ing degeneration.  The  walls  of  the  blood  vessels  are  thickened  and 
sclerosed.  The  hemorrhagic  focus  is  eventually  absorbed  and  replaced 
either  by  a  scar  or,  as  it  happens  in  large  hemorrhages,  by  a  cyst,  or  a  cavity 
surrounded  by  a  thick  capsule. 

Etiology. — Primary  hematomyelia  may  be  spontaneous  or  traumatic. 

In  the  spontaneous  cases  the  underlying  cause  is  a  state  of  congestion 
of  the  cord.  The  hemorrhage  then  occurs  after  a  great  physical  effort, 
viz.  violent  exercises,  coitus,  paroxysms  of  whooping  cough.  It  has  also 
been  observed  in  purpura,  in  hemophilia,  in  cases  of  suppression  of  menses 
or  of  hemorrhoidal  bleeding,  sudden  decompression  (see  Divers'  Disease). 
Spontaneous  hematomyelia  is  rare. 

Traumatism  is  the  most  frequent  cause  of  hematomyelia.  Fracture, 
dislocation  of  the  vertebrae,  obstetrical  intervention,  dystocia  forced, 
inclination  of  the  head,  a  blow  or  a  fall  on  the  back,  a  fall  on  the  feet,  are 
the  usual  causes.  In  such  cases  there  may  be  a  predisposing  factor,  such 
as  preexisting  vascular  changes. 

Symptoms. — The  onset  is  exceptionally  sudden  (Vulpian's  spinal 
apoplexy),  sometimes  with  loss  of  consciousness.  The  patient  experiences 
a  sharp  pain,  the  seat  of  which  depends  upon  the  level  of  the  injured 
vertebras.  The  patient  falls  and  immediately  paralytic  symptoms, 
disturbances  of  sensations  and  of  the  sphincters  appear.  Sometimes  these 
symptoms  appear  only  a  few  hours  after  the  onset,  but  pain  in  the  spine  is 
always  present.  The  lower  extremities  are  usually  involved,  but  accord- 
ing to  the  seat  of  the  lesion  there  may  be  paralysis  of  all  four  extremities 
or  only  of  the  arms.  The  paralysis  is  flaccid,  when  the  lesion  is  in  the 
dorso-lumbar  segment  of  the  cord;  if  it  is  in  the  cervical  cord,  the  paralysis 
of  the  lower  extremities  will  be  spastic.  The  reflexes  are  exaggerated  in 
the  spastic  palsy,  abolished  in  flaccid  paralysis.  The  reflexes  as  a  rule  are 
abolished  immediately  after  the  onset,  irrespective  of  the  level  at  which  the 
injury  occurs. 

The  muscles  gradually  undergo  atrophy. 

The  sensory  disturbances  at  the  beginning  will  consist  of  a  numbness 
and  tingling  in  the  affected  limbs.  Objective  sensory  disturbances  are 
always  present.  They  consist  of  a  marked  diminution  of  all  forms  of  sen- 
sations or  of  a  complete  anaesthesia  of  the  affected  limbs.  Sometimes  a 
syringomyelic  sensory  dissociation  may  be  evident:  it  consists  of  a  loss  of 


HEMATOMYELIA 


28l 


n 

(T        ....              ,     XX 

1 

III.              ^>-?b 

! 

CO 

Will. -^^f\ 

II 

III  _ 


iV.  £0  rectus  lateralis 

11  to  rectus  antic,  minor 

Anastomosis  with  hypoglossal 

Anastomosis  with  pneumogastric 

N.  to  rectus  antic. major. 

N.  to  mastoid  region. 

Great  auricular  n. 

Transverse  cervical  n. 
==}JT.  to  Trapezius,  Aug.  Scap.  and  Rhomboid. 

Supra  clavicular  n. 

Supra-acromial  n. 

Phrenic  n. 

N.  to  levator  ang.  scap. 

Jf.  to  rhomboid 

Subscapular  n. 

Subclavicular  n. 


N.  to  peetoralis  major. 


.Circumflex  n. 

Musculo-cutaneous  n. 

Median  n. 

Radial  n. 
Ulnar  n. 

Internal  cutaneous  n. 

Small  internal  cutaneous  n. 


Iliohypogastric  n. 

Ilio-inguinal  n. 


..External  cutaneous  n. 
Genito-crural  n. 


Anterior  crural  n. 
Obturator  n. 


N.  to  levator  ani 

A.  to  obturator  int. 

JV.  to  ophincter  ani 

Coccygeal  n._ 


Superior  gluteal  n. 


JV.  to  pyriformis 

N.  to  gemellus  super. 

_AT.  to  gemellus  infer. 

If.  to  guadratus 

Small  sciatic  n. 


Sciatic  n. 

Fig.  92. — Relation  of  Segments  of  Spinal  Cord  and  Their  Nerve  Roots  to  the  Bodies 
and  Spines  of  the  Vertebra.     {After  Dejerine  and  Thomas.) 


282 


HEMATOMYELIA 


diminution  of  sense  of  pain  and  temperature  and  preservation  of  the  sense 
of  touch.     According  to  Minor,  the  latter  occurs  in  central  hematomyelia. 

A  very  important  and  early  symptom  which  persists  is  a  paralysis  of 
the  sphincters  of  the  bladder  and  rectum  (constipation  and  retention  of 
urine).  There  is  usually  a  rise  of  temperature  at  the  beginning,  but  it  goes 
down  to  normal  in  a  few  days. 

If  death  does  not  occur  at  the  end  of  a  few  days,  all  the  symptoms  begin 
to  improve  and  some  of  them  may  even  disappear.  The  flaccid  paralysis 
becomes  spastic.     Then  the  reflexes  are  increased.     Ankle-clonus,  Babin- 


Fig.  93. 


-Brown-Sequard's  Paralysis  Following  a  Gunshot  Fracture  of  the  Right 
Fifth  Cervical  Vertebra. 


Paralysis  of  the  right  upper  and  lower  limbs  and  sensory  disturbances  on  the  left.  The 
black  indicates  thermoanesthesia  and  analgesia;  the  portion  shaded  in  lines — hypalgesia  and 
thermo-hypsesthesia. 


ski's  sign,  paradoxical  reflex,  Oppenheim's  reflex,  make  their  appearance 
in  the  lower  extremities.  Muscular  atrophy  with  reactions  of  degeneration 
develop  rapidly.  When  the  hemorrhage  is  in  the  cervical  cord,  the  muscu- 
lar atrophy  of  the  upper  extremities  is  of  Aran-Duchenne's  type.  The 
sensory   disturbances  usually   disappear,   but   sometimes   syringomyelic 


HEMATOMYELIA  283 

dissociation  persists.  The  condition  of  the  sphincters  improves  to  some 
extent. 

In  hematomyelia  of  the  lowest  cervical  segment,  in  addition  to  the 
spastic  paralysis  and  muscular  atrophy  of  the  upper  extremities  and  sen- 
sory disturbances  of  syringomyelic  type,  there  are  also  oculo -pupillary 
disturbances,  viz.  myosis,  narrowness  of  the  palpebral  fissure.  When 
only  the  eighth  cervical  and  the  first  dorsal  segments  are  involved,  the 
sensory  disturbances  occupy  a  longitudinal  band  at  the  inner  border  of  the 
entire  limb  and  the  atrophy  is  limited  to  the  muscles  of  the  hands.  When 
the  seventh,  sixth  and  fifth  cervical  segments  are  affected,  the  sensory  dis- 
turbances are  on  the  external  border  or  on  the  anterior  surface  of  the  limb; 
the  atrophy  affects  the  muscles  of  the  shoulder,  arm  and  forearm.  When 
the  hemorrhage  occurs  in  the  upper  cervical  segment,  paralysis  of  the 
phrenic  nerve  and  of  the  diaphragm  and  consequently  difficulty  of  respira- 
tion will  ensue.  In  hemorrhages  of  the  thoracic  cord  there  will  be  spastic 
paralysis  of  the  lower  limbs,  paralysis  of  the  abdominal  muscles,  and  in- 
volvement of  the  sphincters. 

When  a  hemorrhage  occurs  in  the  lowest  part  of  the  cord,  viz.  in 
the  conus  medullaris,  there  will  be  a  special  symptom-group  which  will 
be  described  in  "Diseases  of  the  Conus." 

If  the  seat  of  the  hemorrhage  is  in  one  half  of  the  cord,  the  clinical  pic- 
ture will  be  that  of  Brown-Sequard's  paralysis.  It  consists  of  a  loss  or 
diminution  of  sensations  on  the  side  opposite  the  lesion  and  of  a  motor 
paralysis  on  the  side  of  the  lesion;  these  motor  and  sensory  disturbances 
are  distributed  in  the  portions  of  the  body  below  the  lesion. 

Course,  Termination,  Prognosis. — Death  may  occur  in  a  few  hours  or 
days.  In  the  majority  of  cases  improvement  follows.  The  prognosis  can- 
not be  made  before  the  acute  stage  has  subsided.  When  the  hemorrhage 
is  in  the  cervical  cord,  the  outlook  is  very  serious  because  of  the  involvement 
of  the  respiratory  centers.  Disturbance  of  the  sphincters  may  lead  to 
cystitis  and  ascending  nephritis.  Even  in  the  most  favorable  cases 
complete  recovery  can  never  be  expected. 

Diagnosis. — In  Syringomyelia  paralysis,  muscular  atrophy  and  sensory 
dissociation  are  characteristic,  but  a  sudden  onset  with  a  very  marked 
paraplegia  are  only  met  with  in  hematomyelia. 

Acute  Myelitis  will  be  recognized  by  absence  of  atrophy,  and  if  the 
latter  is  present,  it  is  usually  slight.  Besides,  in  myelitis  the  onset  is  not 
so  abrupt  and  the  paralytic  symptoms  are  predominant. 

In  meningeal  hemorrhages  there  is  a  very  intense  pain  in  the  back  along 
the  nerves,  the  spinal  muscles  are  rigid,  but  no  sensory  dissociation. 

Treatment. — At  the  onset  absolute  rest  is  necessary.     Immobilization 


284  CAISSON   DISEASE 

of  the  body  is  advisable  in  order  to  avoid  another  hemorrhage.  The  patient 
should  lie  on  his  side.  Counter-irritants  are  to  be  avoided,  as  they  may 
lead  to  ulcerations.  Every  effort  should  be  made  to  avoid  secondary 
infections.  Ergot  had  been  recommended.  In  the  chronic  stage  massage, 
warm  baths  and  systematic  exercises  (see  Treatment  of  Spastic  Paraplegia) 
should  be  applied. 

HI.  Caisson  Disease  (Divers'  Paralysis) 

Divers  or  workers  in  caissons,  being  under  the  influence  of  high  at- 
mospheric pressure  while  at  work,  present  nervous  disturbances  when  they 
return  to  the  surface. 

Pathology. — The  most  constant  microscopical  changes  found  in  al- 
most every  case  are:  congestion  of  brain  and  cord  and  internal  organs  in 
acute  cases,  softening  in  chronic  cases.  In  the  incurable  cases  of  long  stand- 
ing, in  which  the  condition  remains  permanent  until  death,  lesions  of 
typical  chronic  myelitis  are  the  usual  findings.  In  one  case  that  came 
under  my  observation  the  patient  lived  three  months.  The  autopsy 
showed  softened  areas  in  the  gray  matter  and  in  the  posterior  and  lateral 
columns.  Ascending  and  descending  degenerations  could  be  traced. 
Leyden  reported  a  rupture  of  the  dorsal  cord. 

Etiology.— The  immediate  cause  of  the  disorder  of  the  nervous  system 
is  the  lessened  atmospheric  pressure  when  the  workers  return  from  the 
caisson,  but  the  primary  factor  is  the  increased  atmospheric  pressure 
beneath  the  surface. 

Symptoms. — Shortly  after  the  return  to  the  surface  and  after  a  pro- 
dromal stage,  consisting  of  pain  more  or  less  severe  in  the  large  joints 
in  the  muscles,  especially  in  those  of  the  back,  also  in  the  epigastrium  and 
sometimes  over  the  entire  body,  a  paralysis  occurs.  The  most  frequent 
form  of  this  is  paraplegia,  but  sometimes  hemiplegia  is  observed.  The 
pain  is  of  a  neuralgic  character  and  it  may  be  so  severe  that  it  doubles  up 
the  patient,  hence  the  common  term  "  the  bends."  The  paralysis  attacks 
more  frequently  the  lower  extremities  than  the  upper.  Sensations  are  always 
disturbed:  there  may  be  total  anaesthesia  in  the  limbs  and  at  the  same  time 
intense  pain.  The  onset  and  the  character  of  the  paralysis  is  very  similar 
to  that  of  transverse  myelitis.  If  we  take  into  consideration  the  frequent 
involvement  of  the  sphincters  (retention  and  constipation)  and  the  sensory 
disturbances,  the  resemblance  to  myelitis  will  be  complete.  The  involve- 
ment of  the  motor  and  sensory  apparatuses  may  be  complete  or  only 
partial  or  else  unequally  distributed.  There  may  be  no  relationship 
between  the  objective  sensory  disturbances  and  the  degree  of  paralysis; 


CAISSON  DISEASE  285 

either  may  be  affected  without  the  other.  In  some  cases  there  may  also 
be  vertigo,  headache,  vomiting,  slight  confusion,  convulsions  and  double 
vision.  Prostration  is  present  in  severe  cases.  In  fatal  cases  deep  coma, 
irregular  respiration  and  symptoms  of  cardiac  paralysis  announce  approach- 
ing death.  As  a  not  infrequent  occurrence  may  be  mentioned  small 
perforations  of  the  ear-drums  or  such  a  congestion  of  the  inner  ear  as  to 
give  the  picture  of  Meniere's  disease.  Aphasia  has  been  occasionally 
observed.     Ecchymosis  of  the  skin  is  also  an  occasional  occurrence. 

Pathogenesis. — There  are  two  views  for  the  explanation  of  the  symp- 
toms. According  to  one  of  them,  the  so-called  gaseous  theory,  the  blood 
while  under  high  pressure  becomes  overcharged  with  gas  (oxygen  and 
carbonic  acid).  When  the  surface  is  reached,  the  gas  attempts  to  escape 
through  the  lungs.  In  the  meantime  the  superfluous  gas  circulates  in 
the  blood  in  bubbles,  and  may  either  form  emboli  or  escape  through  the 
vessel  walls  into  the  surrounding  tissues  and  consequently  produce  con- 
siderable pressure.  The  spinal  cord  suffers  the  most,  because  besides 
being  situated  in  a  hermetically  closed  cavity,  it  has  a  slow  return  circula- 
tion (because  of  a  large  number  of  plexuses).  According  to  the  other  view, 
there  is  a  congestion  followed  by  stasis.  The  high  pressure  drives  the 
blood  from  the  periphery  to  the  internal  organs,  especially  to  the  nervous 
system.  The  blood  vessels  of  the  latter  having  no  support  from  counter- 
pressure  remain  dilated.  A  paralysis  of  their  walls  takes  place.  When 
the  atmospheric  pressure  is  diminished,  the  paralyzed  vessels  cannot 
follow  and  stasis  will  be  the  result. 

Prognosis. — An  attack  may  last  from  several  hours  to  six  days. 
When  the  symptoms  are  pronounced,  death  usually  ensues.  When 
the  paralytic  and  other  symptoms  are  mild,  improvement  and  even 
recovery  may  follow.  In  some  cases  the  myelitic  symptoms  persist 
indefinitely. 

Treatment- — It  is  that  of  myelitis  (see  above).  Prophylaxis  is  the 
most  important  part  of  the  treatment.  Bad  physical  health,  diseases  of 
the  kidneys  and  heart,  alcoholism,  obesity  and  hunger  are  all  contra- 
indications for  subjecting  one's  self  to  high  atmospheric  pressure.  The 
return  to  the  surface  should  be  done  in  caissons  supplied  with  locks,  so 
that  the  reduction  of  pressure  be  made  very  gradually.  Haldane  and 
Boycott  {Jour,  of  Hygiene,  Cambridge,  1908)  have  laid  out  the  following 
rules  for  gradual  decompression:  when  a  diver  has  been  working  at  a 
depth  of  144  feet  for  ninety  minutes,  he  should  ascend  at  once  to  50  feet, 
then  rest  for  ten  minutes.  From  that  time  on  he  should  come  up  10  feet 
four  times  in  succession  with  intervals  of  ten,  twenty,  thirty  and  thirty- 
five  minutes,  after  which  he  leaves  the  water. 


SYRINGOMYELIA 


IV.  Syringomyelia 


Syringomyelia  is  characterized  by  a  formation  of  a  cavity  or  cavities 
in  the  spinal  cord. 

Pathology. — The  macroscopical  aspect  of  a  syringomyelic  cord  is 
often  quite  characteristic.  It  may  be  flat,  soft  or  fluctuating.  Its  cervical 
portion,  the  usual  seat  of  the  lesion,  is  unduly  enlarged.  A  transverse 
section  will  reveal  the  presence  of  a  cavity ;  the  latter  may  be  single  or 
multiple.  It  is  mostly  situated  in  the  cervical  segment,  it  may  also 
occupy  the  entire  length  of  the  cord.  Most  frequently  it  is  found  in  the 
posterior  commissure  and  posterior  cornua.  In  some  cases  it  extends 
so  far  backwards  that  it  destroys  the  posterior  roots  at  their  entrance 
into   the  cord.     The  white  matter  (lateral  columns)  becomes  involved 


Fig.  94. — Hydromyelia  of  the  Upper  Dorsal  Cord.     (Striimpell.) 

when  the  cavity  has  destroyed  the  gray  substance.  The  cavity  may 
extend  into  the  medulla.  A  large  cavity  may  open  into  the  central  canal. 
Sometimes  the  entire  pathological  process  consists  of  a  primary  enlarge- 
ment of  the  central  canal.     The  latter  constitutes  hydromyelia. 

The  contents  of  syringomyelic  cavities  is  a  fluid  analogous  to  the 
cerebro-spinal  fluid.  Microscopically  a  gliomatous  formation  is  noticed, 
in  the  midst  of  which  lies  the  cavity.  This  tissue  consists  of  glia  cells  and 
glia  fibers. 

The  cavity  is  due  to  a  softening  and  collapse  of  the  center  of  the 
gliomatous  tissue.  The  normal  nervous  elements  affected  by  the  latter 
are  naturally  destroyed.  The  tracts  of  nerve-fibers  degenerate  according 
to  their  directions.  Cavities  may  be  also  the  result  of  inflammatory 
processes  within  the  cord  or  of  hematomyelia  (see  this  chapter) .  In  such 
cases,  instead  of  the  characteristic  elements  of  glioma,  the  cavity  is  sur- 
rounded by  sclerosed  tissue.  These  two  forms,  while  clinically  they  may 
present  the  typical  symptoms  of  the  disease,  are  nevertheless  not  the 


SYRINGOMYELIA  287 

true  syringomyelia,  which  is  essentially  a  gliomatosis  and  has  for  basis 
very  probably  a  developmental  anomaly:  the  nests  of  glia  cells  left  in  the 
central  canal  begin  through  some  cause  (trauma  or  others)  to  proliferate 
and  lead  to  new  formations. 

Etiology. — Trauma  apparently  plays  an  important  role,  as  there  are  a 
number  of  cases  on  record,  in  which  the  symptoms  begin  to  develop  shortly 
after  the  shock.  Guillain  called  attention  to  this  fact  that  an  infected 
wound  may  be  the  cause  of  syringomyelia  by  means  of  an  ascending 
neuritis.  Exposure  to  cold,  exhaustion  are  also  mentioned  by  some 
writers  among  the  causes  of  the  disease.  The  consensus  of  opinion  is 
that  a  congenital  defect  of  the  central  canal  is  the  most  frequent  cause. 
Most  frequently  the  disease  begins  at  the  age  of  between  ten  and  thirty. 
There  are  also  cases  with  an  onset  in  infancy.  Males  are  more  frequently 
affected  than  females. 

Symptoms. — They  are:  sensory,  trophic  and  motor. 

Sensory. — They  constitute  the  sensory  dissociation  characteristic  of 
the  affection  It  consists  of  loss  of  sense  of  pain  and  temperature  with 
preservation  of  the  sense  of  touch.  The  involvement  of  temperature  sense 
(thermoanesthesia)  is  a  very  grave  sign.  The  patients  often  being  un- 
aware of  it.  burn  their  hands.  In  one  of  my  recent  cases  the  hands  and 
forearms  were  literally  covered  with  cicatrices  as  a  result  of  unnoticed 
burns.  It  is  only  accidentally  the  patient  would  notice  that  her  hands 
came  in  contact  with  fire  or  a  hot  stove.  Application  of  ice  to  the  hands 
did  not  give  her  the  slightest  sensation  of  cold;  she  felt  only  the  contact  of 
a  solid  object. 

The  loss  of  pain-sense  (analgesia)  is  usually  of  the  same  intensity  as  that 
of  temperature.  The  patient  just  mentioned  developed  a  cellulitis  of  the 
palm  of  one  hand  and  at  no  time  she  experienced  pain.  An  incision  was 
made  by  the  surgeon  without  a  general  or  local  anaesthetic,  the  wound 
was  cauterized  and  no  pain  was  felt  by  the  patient.  She  also  had  a  dis- 
located shoulder  on  one  side  and  she  could  not  tell  how  and  when  it  appeared. 
A  close  questioning  elicited  a  history  of  a  fall.  She  therefore  did  not  feel 
the  pain  at  the  time  of  the  dislocation.  The  characteristic  sensory 
dissociation  is  not  absolute  in  every  case.  Pain  and  temperature  senses 
may  be  only  diminished,  or  one  may  be  more  or  less  pronounced  than  the 
other.     The  tactile  sense  in  advanced  cases  may  sometimes  be  also  affected. 

It  must  be  also  mentioned  that  sometimes  the  thermal  sense  may  be 
perverted,  viz.  heat  is  taken  for  cold  and  cold  for  heat.  Another  charac- 
teristic feature  of  the  sensory  disturbances  lies  in  their  radicular  distribu- 
tion, viz.  longitudinal  or  parallel  to  the  limbs.  The  upper  extremities 
and  the  trunk  are  more  frequently  involved  than  the  lower  limbs. 


288  SYRINGOMYELIA 

Subjective  sensory  disturbances  are  usually  present.  Pain  is  frequent, 
especially  at  the  beginning.  The  sense  of  pressure  is  diminished.  Astere- 
ognosis  is  sometimes  observed. 

The  existence  of  the  sensory  dissociation  can  be  explained  by  this 
anatomical  fact,  that  Gowers'  tract,  which  contains  fibers  for  conducting 
pain  and  temperature,  originate  in  the  cells  of  Clarke's  column  of  the 
opposite  side.  As  they  cross  the  median  line  they  are  interrupted  by  the 
syringomyelic  cavity,  the  usual  seat  of  which  is  near  the  central  canal  (see 
Anatomy) . 

Trophic. — Muscular  atrophy  is  almost  constant.  The  cause  of  it  is 
the  involvement  of  the  anterior  cornua  by  the  cavity.     It  has  usually 


Fig.  95. — Syringomyelia.     Deformity  of  Hands  and  Fingers,   Cicatrices   on  Some 

Fingers  as  a  Result  of  Self  Amputation  or  Painless  Ulcers. 

(Edematous  Hands. 


the  form  of  Aran-Duchenne's  type  (see  Progressive  Muscular  Atrophy). 
Here  we  find  the  typical  onset  in  the  small  muscles  of  the  hands,  the  claw- 
like hand,  the  preacher's  hand  (Charcot).  The  latter  is  due  to  overac- 
tion  of  the  extensors  of  the  forearm,  while  the  muscles  supplied  by  ulnar 
and  median  nerves  are  paralyzed.  The  remaining  muscles  of  the  upper 
extremities  as  well  as  those  of  the  shoulders  follow  the  atrophy  of  the 
muscles  of  the  hands.     Later  on  the  muscles  of  the  back,  intercostal  and 


SYRINGOMYELIA  289 

abdominal  and  finally  those  of  the  lower  limbs  undergo  atrophy.  Deform- 
ities of  the  feet  follow.  The  atrophy  does  not  always  follow  the  regularity 
just  mentioned.  There  are  great  variations  in  its  distribution.  The 
character  of  this  distribution  is  chiefly  radicular. 

Fibrillary  contractions  in  the  affected  muscles  are  constant.  Reactions 
of  degeneration  or  only  a  quantitative  diminution  of  electrical  contractility 
may  be  present. 

Besides  the  muscles  other  tissues  suffer  in  their  nutrition.  The  skin 
may  break  and  leave  sores.  It  may  become  glossy,  covered  with  vesicles 
and  eruptions,  the  nails  suffer,  the  phalanges  are  deformed.  Morvan's 
disease,  which  is  characterized  by  painless  panaritiae,  is  a  variety  of  syrin- 
gomyelia: the  tendons  and  terminal  phalanges  become  necrosed,  are 
gradually  eliminated  and  slowly  a  cicatrix  is  formed.  During  this  entire 
process  there  is  no  pain.  The  perforating  ulcer  on  the  feet  is  frequent  in 
syringomyelia.  Raynaud's  disease  is  sometimes  observed,  although  it 
never  reaches  the  phase  of  gangrene.  Marinesco  described  an  oedematous 
hand  (main  succulente),  which  does  not  pit  on  pressure,  as  pathognomonic 
of  the  disease  (Fig  95). 

Arthropathies  are  quite  frequent.  It  may  be  only  hydarthrosis  or  a 
destruction  of  the  epiphyses  with  subsequent  dislocation. 

The  bones  also  suffer  in  their  nutrition.  Spontaneous  fractures 
occur.  Formation  of  a  callus  is  slow  and  difficult.  Scoliosis  associated 
with  kyphosis  is  not  infrequent. 

Vasomotor  disturbances  consist  of  cyanosis  of  the  extremities,  oedema, 
hyperhidrosis. 

Motor. — Paralytic  symptoms  develop  slowly  and  progressively. 
Spastic  paralysis  is  a  frequent  symptom.  In  some  cases  it  is  very  pro- 
nounced and  contractures  deform  the  trunk  and  the  extremities.  In 
such  cases  the  reflexes  are  exaggerated  and  the  toe  phenomenon  will  be 
present.  When  the  posterior  columns  become  involved,  tabetic  symptoms 
will  be  manifested,  viz.  ataxia,  Romberg's  sign,  loss  of  reflexes.  Various 
oscillatory  movements,  tremors,  choreiform  movements  have  been  observed 
in  some  cases. 

The  sphincters  are  usually  not  involved. 

Ocular  symptoms  are  present  when  the  lesion  is  in  the  lower  cervical 
region,  and  in  the  first  dorsal  segment:  myosis,  retraction  of  the  eyeball, 
narrowing  of  the  palpebral  fissure,  Argyll-Robertson  pupil,  and  defective 
sweating. 

When  the  cavity  extends  to  the  medulla,  bulbar  symptoms  develop. 
Difficulty  of  deglutition,  paralysis  of  the  vocal  cords,  irregular  action  of  the 
heart,  facial  paralysis,  hemiatrophy  of  the  face  and  tongue,  ocular  palsies 
*9 


29O  SYRINGOMYELIA 

with  nystagmus  are  then  observed.  Sometimes  the  lesion  involves  the 
trigeminal  nerve;  anaesthesia  over  its  area  of  distribution  will  be  the 
consequence. 

Course,  Termination,  Prognosis. — The  disease  is  essentially  chronic 
and  its  onset  is  usually  slow.  It  lasts  may  years,  during  which  there  may 
be  periods  of  remission,  but  it  is  inevitably  fatal.  Death  ensues  either 
from  bulbar  involvement  or  from  some  intercurrent  disease  (tuberculosis, 
pneumonia,  etc.). 

Diagnosis. — Progressive  muscular  atrophy  of  spinal  origin  has  the 
same  distribution  of  wasting  as  syringomyelia,  but  in  the  former  the 
characteristic  sensory  disturbance  of  the  latter  is  absent. 

Amyotrophic  lateral  sclerosis  will  be  also  recognized  by  the  absence 
of  sensory  dissociation  and  of  trophic  disturbances. 

Syringomyelia  presents  in  some  cases  tabetic  symptoms  (ataxia,  loss  of 
reflexes,  Romberg's  sign,  arthropathies),  but  the  absence  of  characteristic 
ocular  symptoms,  of  sphincter  disturbances  of  tabes  will  soon  decide  the 
diagnosis. 

Leprosy  is  characterized  by  muscular  atrophy,  sensory  and  trophic 
disturbances.  The  atrophic  hand  of  leprosy  resembles  that  of  syrin- 
gomyelia, but  the  mutilations  of  the  phalanges  in  the  latter  never  reaches  the 
extent  of  those  of  leprosy.  The  mutilated  toes  (rare  in  syringomyelia), 
muscular  atrophy  in  the  lower  limbs  (rare  in  syringomyelia),  absence  of 
scoliosis,  absence  of  increased  reflexes — are  all  in  favor  of  leprosy.  In 
the  latter  disease  sensory  dissociation  is  rare. 

In  Morvan's  disease  the  number  of  painless  panaritise  is  considerable. 
Sensory  dissociation  is  usually  absent. 

Treatment. — It  is  only  symptomatic  The  ulcerations  will  be  treated 
antiseptically.  For  muscular  atrophy  massage  and  electricity  are  advis- 
able Care  should  be  taken  of  the  skin  in  giving  massage,  as  erosions 
are  likely  to  lead  to  ulcerations,  which  are  slow  in  healing  Surgical 
operations  should  be  avoided  Internally  iodides,  arsenic  and  iron  may 
be  given 

During  the  last  few  years  favorable  results  have  been  obtained  from 
treatment  of  Syringomyelia  with  X-rays.  Among  the  most  recent  con- 
tributions to  the  subject  may  be  mentioned  that  of  Allaire  and  Denes 
(Gaz.  Med.  de  Nantes  Nov.,  1912). 

Morvan's  Disease 

Under  this  name  is  desrcibed  an  affection  which  has  been  observed  in 
Normandy  and  which  according  to  some  neurologists  can  be  considered 
as  a  clinical  variety  of  syringomyelia,  in  which,  in  addition  to  the  anaes- 


DISEASES    OF   CONUS   MEDULLARIS 


291 


thesia  and  analgesia,  paretic  symptoms  with  muscular  atrophy,  there 
are  also  multiple  painless  panarities.  However  the  sensory  dissociation 
is  usually  absent.  The  sense  of  touch  is  affected  as  much  as  the  pain  and 
temperature  senses.  The  sensory  disturbances  are  distributed  like  in 
neuritis,  viz.  decreasing  from  the  distal  to  the  proximal  end  of  the  limb. 


VaJlteB/10-5- 


V.  Diseases  of  Conus  Medullaris  and  Cauda  Equina 

The  lower  portion  of  the  spinal  cord,  called  conus  medullaris,  is  sur- 
rounded by  bundles  of  nerve  fibers  coming  from  lumbar  and  sacral  seg- 
ments (cauda  equina)  The  study  of  this 
important  portion  of  the  cord  is  com- 
paratively recent.  Anatomically  the 
conus  corresponds  to  the  second  lumbar 
vertebra  and  its  fllum  terminale  begins  at 
the  level  of  the  middle  of  this  vertebra. 
The  conus  comprises  the  last  three  sacral 
and  coccygeal  segments.  Physiologically 
conus  medullaris  is  a  very  important  por- 
tion of  the  cord.  It  contains  special  and 
independent  centers  for  micturition,  de- 
fecation, erection,  ejaculation  and  for  the 
anal  reflex.  Diseases  of  the  conus  present 
a  special  clinical  picture  which  deserves  a 
separate  description. 

Pathology. — All  possible  lesions  of  the 
spinal  cord  studied  on  the  preceding  pages 
may  also  affect  the  conus.  We  may 
therefore  have  those  of  myelitis,  of  hem- 
orrhage, of  tabes,  etc. 

Etiology. — Trauma  is  the  most  frequent 
of    all    causes.     In   cases   of   fracture  of 

the  lumbar  and  sacral  vertebras,  a  fragment  is  likely  to  press  directly 
upon  the  conus  or  the  roots.  Secondary  myelitis  will  follow.  Trauma 
may  also  produce  a  hemorrhage  in  the  conus.  A  blow  over  the  lum- 
bar region  may  produce  a  traumatic  myelitis  of  the  conus.  An  injury 
below  the  second  lumbar  vertebra  will  damage  only  the  cauda  equina; 
at  the  level  of  the  second  lumbar  will  involve  the  conus  and  the  cauda 
equina.  An  injury  immediately  above  the  second  lumbar  vertebra 
will  damage  the  conus  alone.  The  lowest  part  of  the  spinal  cord  is  not 
infrequently  the  seat  of  tumors.     The  latter  may  originate  in  the  vertebrae 


Fig.  96. — Conus  Medullaris 
(Ajter  Raymond.) 


292 


DISEASES    OF    CONUS    MEDULLARIS 


or  in  the  cutaneous  coverings  or  else  be  primary  (sarcoma,  lymphangi- 
oma, glioma,  gumma).  Abscess  due  to  tuberculosis  of  the  vertebrae 
may  also  involve  the  conus. 

Symptoms.  Conus. — From  the  anatomical  fact  mentioned  above, 
namely  that  the  conus  is  the  center  for  the  third,  fourth  and  fifth  pairs 
of  sacral  nerves  (Fig.  92),  also  from  the  physiological  character  of  the 
conus,  viz.  that  it  contains  special  centers  for  micturition,  defecation,, 
etc.  (see  above),  there  is  no  difficulty  in  presenting  a  clinical  picture  of 


Fig.  97 a.  — Solid  Portion  Thermoanesthesia;  Shaded,  Reversed  Temperature  Sense. 

diseases  of  the  conus.  The  symptoms  therefore  are  (Figs.  97a,  97,  98): 
1.  Anaesthesia  or  sensory  dissociation  of  the  external  genital  organs, 
perineum,  anus,  inferior  gluteal  region  and  the  postero-superior  area  of 
the  thighs  (saddle-shaped  anaesthesia).  It  may  also  extend  to  the  mucous 
membrane  of  the  genito-urinary  apparatus.  The  tenderness  of  the  tes- 
ticles is  intact. 

\      2.  Paralysis  of  the  bladder  and  rectum.     There  may  be  incontinence 
or  retention. 

3.  Involvement   of    the    sexual    function    and    of    the    sensation   of 
ejaculation. 


DISEASES    OF   CONUS   MEDULLARIS 


293 


4.  Pain  is  a  frequent  but  not  constant  phenomenon.  If  it  is  present, 
it  may  be  of  great  severity.  As  to  the  motor  power  of  the  lower  extrem- 
ities it  is  normal.     The  reflexes  are  equally  unaffected. 

The  onset  of  these  symptoms  is  sudden  in  traumatic  cases,  slow  in  cases 
of  tumors.  As  the  conus  is  immediately  surrounded  by  the  nerve  bundles 
(cauda  equina),  a  lesion  of  one  is  bound  to  involve  the  other  and  the  symp- 
tomatology therefore  is  complex. 


Fig.  97. — Solid  Portion,  Analgesia. 


Cauda  Equina. — If  a  tumor  is  situated  below  the  conus,  the  main  symp- 
toms will  be  those  of  involvement  of  the  roots,  viz.  pain  and  objective 
sensory  disturbances.  The  pain  is  intense  in  the  lumbo-sacral  region 
and  in  the  limbs,  chiefly  in  the  region  of  both  sciatic  nerves;  it  is  constant 
and  increased  upon  the  slightest  movement.  The  objective  sensory  dis- 
turbances may  be  only  anaesthesia.  They  occupy  the  lower  extremities 
beginning  from  the  lower  two-thirds  of  the  thighs  downward,  the  perineum, 
anus  and  the  genito-urinary  organs.     There  is  anaesthesia  of  the  mucous 


294 


DISEASES    OF   CONUS    MEDULLARIS 


membranes  of  the  urethra,  bladder,  vulva,  vagina.  The  paralysis  is 
flaccid  at  the  beginning  and  during  the  entire  course  of  the  diesase. 
There  is  a  high-steppage  gait.  Muscular  atrophy  appears  early  in  the 
lower  limbs.  Reactions  of  degeneration  are  present.  The  reflexes  Achilles, 
plantar  and  anal  are  abolished,  but  the  knee-jerks  are  intact.  The 
sphincters  are  disturbed.     Bed-sores  are  frequently  present. 


Perineum. 
Splid  portion,  Anesthesia  to  touch,  pain  and  temperature. 


Perineum. 
Solid  portion,  thermo-anesthesia;  shaded  portion,  reversed  temperature  sense. 

Fig.  98. 

Course,  Prognosis. — In  traumatic  cases  death  may  follow  the  injury, 
but  usually  the  condition  becomes  chronic.  The  outlook  is  never  favor- 
able, but  much  better  in  lesions  of  the  cauda  equina  than  of  the  conus. 
The  involvement  of  the  sphincters  is  permanent  and  eventually  leads  to 
the  consequences  described  in  myelitis. 

Diagnosis. — It  is  very  important  to  localize  the  lesion  in  the  conus  or 
in  the  cauda  equina.  In  the  first  case  operative  procedures  are  impossible, 
while  in  the  second  case  they  are  indicated. 

Lesions  of  cauda  equina  are  usually  very  painful,  the  pain  radiates  to- 
ward the  legs,  the  symptoms  develop  gradually.     The  pain  is  present  be- 


DISSEMINATED    SCLEROSIS  295 

low  the  second  lumbar  vertebra;  flexion  of  the  thigh  over  the  pelvis  while 
the  leg  is  extended  provokes  pain.  The  function  of  the  bladder  and 
rectum  improves.  On  the  other  hand  pain  above  the  second  lumbar  verte- 
bra, well-marked  anaesthesia  and  especially  sensory  dissociation,  exceed- 
ingly slight  or  no  improvement  at  all  in  the  function  of  the  sphincter — 
are  all  symptoms  of  the  conus. 

Treatment. — Not  much  can  be  expected  from  external  applications,  as 
counter-irritation,  revulsion  in  traumatic  cases.  The  rational  treat- 
ment is  surgical  intervention.  In  cases  where  hemorrhage  is  suspected,  a 
puncture  in  the  spinal  canal  with  evacuation  of  its  contents  may  give 
immediate  good  results.  In  cases  of  fracture  prompt  removal  of  fragments 
is  urgent.  Tumors  must  be  equally  removed.  Before  an  operation  is 
undertaken,  a  detailed  study  of  the  case  must  be  made  as  to  the  localiza- 
tion of  the  sensory  and  other  disturbances  in  order  to  determine  the  exact 
field  of  the  operation  (Fig.  92). 

Epiconus 

In  1900  Minor  called  attention  to  lesions  of  that  portion  of  the  spinal 
cord  which  lies  immediately  above  the  conus  (epiconus) .  It  is  frequently 
involved  in  traumatisms  and  it  presents  a  special  symptomatology,  viz. 
(1)  integrity  of  knee-jerks,  (2)  integrity  of  the  sphincters  of  bladder  and 
rectum,  (3)  loss  of  Achilles  reflex,  (4)  serious  and  protracted  paralysis  of 
the  external  popliteal  nerve. 

Frequently  lesions  of  the  conus  and  epiconus  are  associated  and  not 
rarely  the  cauda  equina  is  simultaneously  involved.  The  special  symptoms 
characteristic  of  each  of  these  portions  will  enable  one  to  make  a  diagnosis 
of  the  complex  cases  as  well  as  when  each  of  them  is  individually 
affected. 

VI.  Disseminated  or  Multiple  Cerebro-spinal  Sclerosis 
(Insular  Sclerosis) 

Pathology. — The  disease  is  characterized  by  islets  of  sclerosis  dis- 
seminated throughout  the  entire  central  nervous  system.  They  can  be 
seen  also  in  some  of  the  cranial  nerves,  viz.  optic,  olfactory  and  trigeminal. 
On  transverse  section  yellowish  spots  of  various  sizes  can  be  seen  even  with 
a  naked  eye.  Their  distribution  is  very  irregular,  they  do  not  show  any 
special  predilection  for  any  particular  tract,  but  they  are  frequently  found 
in  the  white  substance  of  the  cord,  brain  and  cerebellum,  in  the  anterior 
portion  of  the  pons  and  in  the  pyramids  of  the  medulla.     Histologically 


296 


DISSEMINATED    SCLEROSIS 


the  sclerosed  patches  present  a  characteristic  picture:  the  myelin  is  de- 
stroyed, but  the  axis-cylinders  are  remarkably  preserved.  The  latter  fact 
is  probably  the  reason  of  absence  of  secondary  degeneration.  Sometimes 
the  axis-cylinders  are  swollen,  but  they  rarely  disappear,  except  in  very  old 
cases.  The  neuroglia  tissue  in  the  diseased  area  proliferates  abundantly. 
The  blood  vessels  of  sclerosed  patches  are  sometimes,  but  not  always, 
found  altered  (endo-  and  periarteritis)  The  cells  are  usually  spared.  As 
to  the  origin  of  the  sclerotic  process,  some  believe  that  proliferation  of  the 
neuroglia  is  the  initial  process,  to  others  the  initial  lesion  is  a  myelitis: 
the  nerve-fibers  are  primarily  affected  and  the  neuroglia  secondarily 


4 


Fig.  99. — Sclerosis  op  Pyramidal,  Gowers'  and  Direct  Cerebellar  Tracts.     {Original.) 


Symptoms. — In  view  of  the  disseminated  character  of  the  sclerotic 
lesions,  the  clinical  picture  naturally  varies  from  one  case  to  another  and 
depends  upon  the  seat  of  the  sclerotic  islets.  If  the  lesion  is  in  the  poste- 
rior columns,  symptoms  of  tabes  will  be  present.  If  the  lateral  motor  col- 
umns are  affected,  spastic  paraplegia  will  be  the  symptom.  If  the  postero- 
lateral columns  are  involved,  ataxic  paraplegia  will  be  the  result.  If 
Gowers'  tract  is  diseased,  symptoms  of  syringomyelia  will  be  observed. 
If  the  anterior  cornua  suffer,  muscular  atrophy  will  develop.  If  the  inter- 
nal capsule  has  a  plaque  of  sclerosis,  hemiplegia  will  follow 

As  the  essential  feature  of  the  sclerotic  process  is  its  dissemination  in 
various  portions  and  at  various  levels  of  the  central  nervous  system,  a 
combination  of  symptoms  of  all  the  diseases  mentioned  is  expected.     In 


DISSEMINATED    SCLEROSIS 


297 


fact  there  are  several  clinical  forms  of  multiple  sclerosis.  They  depend 
upon  the  predominance  of  one  or  another  group  of  symptoms 

There  is,  however,  a  series  of  symptoms  which  are  very  frequently 
present  in  this  disease  and  without  which  a  diagnosis  of  multiple  sclerosis 
is  almost  impossible.  If  they  are  not  all  always  present,  at  the  same  time 
some  of  them  at  least  will  help  to  decide  the  diagnosis.     They  are: 

1.  Tremor. — It  is  of  a  special  character:  it  appears  only  upon  a  volun- 
tary act.  It  is  an  "intention  tremor."  Should  the  patient,  for  example, 
attempt  to  carry  a  glass  of  fluid  to  his  mouth,  he  is  bound  to  spill  it,  and 
the  nearer  he  gets  it  to  his  mouth  the  more  the  tremor  will  be  pronounced, 


Fig.  100. — Plaques  or  Sclerosis  in  Pyramids  and  Various  Nuclei.     (Original.) 


so  that  the  glass  will  strike  the  mouth  and  the  teeth  in  all  directions  and 
he  will  fail  to  drink  the  contents.  Writing  is  one  of  the  first  acts  in  which 
tremor  is  manifested:  at  first  all  the  letters  are  irregularly,  unequally 
separated  and  placed  at  different  levels.  In  a  more  advanced  state 
writing  is  illegible  and  then  impossible.  The  legs,  the  trunk  and  the  head 
may  also  be  affected  by  tremor,  but  this  is  more  rarely  met  with.  The 
tremor  may  be  at  first  unilateral  or  predominate  on  one  side,  later  it  becomes 
generalized. 

Not  only  upon  voluntary  acts  irregular  jerky  movements  are  observed, 
but  sometimes  jerky  spontaneous  acts  take  place,  such  as  sudden  crying 
or  laughing.     They  occur  without  any  relation  to  a  preceding  emotion. 

2.  Disturbance  of  Speech. — The  patient  speaks  slowly,  pausing 
between  words  and  between  syllables.  In  advanced  cases  he  accentuates 
each  syllable.  It  is  a  scanning,  syllabic,  staccato  speech.  Besides,  the 
pronunciation  of  certain  letters  is  especially  defective,  viz.  b,  c,  g  and  r. 


298  DISSEMINATED    SCLEROSIS 

As  a  rule  the  first  words  are  pronounced  distinctly,  but  soon  the  speech 
becomes  indistinct.  This  is  probably  due  to  fatigue  of  the  muscles  con- 
cerned in  speech. 

3.  Nystagmus. — It  consists  of  an  oscillation  of  the  eye  globes,  when 
the  patient  turns  his  eyes  to  an  extreme  position  laterally  or  vertically. 
Lateral  nystagmus  is  more  frequent  than  vertical.  In  advanced  cases 
the  least  movement  of  the  eye  globes  will  produce  a  nystagmus. 

4.  Disturbance  of  Gait. — It  is  most  commonly  paretic  and  spastic. 
As  soon  as  the  patient  commences  to  walk,  the  lower  limbs  become  con- 
tracted, the  legs  extend  over  the  thighs,  the  feet  over  the  legs,  and  adduc- 
tion is  extreme.  Moreover  the  patient  has  a  tendency  to  walk  on  his 
toes,  he  topples  over  when  he  walks  quickly.'  Sometimes  there  may  be 
a  gait  which  is  spastic  and  ataxic  at  the  same  time.  The  patient  stands 
with  his  feet  widely  separated  and  soon  as  he  attempts  to  walk,  ataxia 
is  evident  and  there  is  a  tendency  to  fall.  Incoordination,  if  present, 
is  of  a  cerebellar  type  (titubation)  (see  Cerebellar  Diseases).  Oppenheim 
speaks  also  of  an  oscillating  gait,  characterized  by  a  generalized  tremor 
as  soon  as  the  patient  takes  the  first  step.  In  advanced  cases  the  paralysis 
and  spasticity  may  be  so  pronounced  that  walking  is  impossible. 

5.  Reflexes  are  in  the  majority  of  cases  increased  in  all  four  extremi- 
ties. Ankle-clonus,  Babinski's  sign,  Oppenheim's  and  paradoxical 
reflexes  may  be  present.     In  some  cases  the  knee-jerks  may  be  abolished. 

The  five  symptoms  just  described,  and  especially  the  first  three,  are 
almost  always  present  in  disseminated  sclerosis.  There  are  a  few  others 
which,  while  not  very  frequent,  are  met  with  in  quite  a  large  number  of 
cases.     They  are: 

1.  Visual. — A  partial  optic  atrophy  is  the  most  frequent.  It  may  be 
unilateral  or  bilateral.  In  the  earlier  stages  of  the  disease  there  is  only 
pallor  of  the  optic  nerves,  which  is  unequally  distributed  in  both  eyes. 
A  progressive  diminution  of  visual  acuity,  contraction  of  the  visual  field, 
central  scotoma  for  certain  colors  are  the  other  ocular  changes.  Transi- 
tory visual  defect  in  one  or  both  eyes  is  quite  common.  Occasionally 
palsies  of  ocular  muscles  occur.  The  sixth  nerve  is  more  frequently 
involved  than  the  third.  The  reflexes  of  the  pupils  are  very  occasionally 
affected. 

2.  Apoplectiform  or  Epileptiform  Seizures.— The  first  are  more  frequent 
than  the  latter.  The  onset  of  hemiplegia  is  accompanied  by  a  loss  of 
consciousness.  The  paralysis  is  usually  transient,  but  it  may  persist. 
When  the  paralysis  persists,  the  disease  is  considered  as  the  hemiplegic 
form  of  multiple  sclerosis.  The  hemiplegia  may  be  complicated  by 
aphasia.     The    apoplectiform    attacks    have    a    tendency  to   repetition. 


DISSEMINATED    SCLEROSIS  299 

This  latter  fact  together  with  the  transient  character  of  the  attacks  makes 
the  resemblance  to  the  attacks  in  paresis  very  striking.  Indeed  errors  of 
such  diagnoses  have  been  made. 

3.  Sensory  Disturbances. — Abnormal  subjective  sensations,  as  ting- 
ling, cramps  and  sharp  pain  of  neuralgic  nature,  are  quite  frequent. 
Headache  is  not  infrequent.  Neuralgia  is  in  the  domain  of  the  fifth  nerve. 
Hypaesthesia  or  hyperesthesia  are  occasionally  observed.  Anaesthesia  is 
rare. 

Among  the  rare  symptoms  of  multiple  sclerosis  may  be  mentioned: 

(1)  Vertigo;  Charcot  observed  it  quite  frequently  at  the  onset;  (2) 
bulbar  symptoms,  viz.  difficulty  of  swallowing,  acceleration  of  pulse, 
atrophy  of  the  tongue,  palsy  with  atrophy  of  facial  muscles;  (3)  muscular 
atrophy  (without  RD.),  (4)  spasmodic  and  involuntary  laughter,  (4) 
mental  feebleness,  (5)  disturbances  of  the  sphincters,  (6)  trophic  disturb- 
ances of  the  skin  and  articulations,  (7)  glycosuria  and  polyuria. 

Forms  of  Multiple  Sclerosis. — Not  all  cases  present  the  typical 
picture  described  above.  In  some  cases  the  chief  symptoms  are  either 
absent  or  very  slightly  manifest.  Charcot  called  such  cases  "formes 
frustes."  According  to  the  seat  of  the  lesions  multiple  sclerosis  may  be: 
cerebral,  spinal  or  cerebro -spinal.  Each  of  these  may  present  varieties 
depending  upon  the  level  at  which  the  lesion  is  found.  Thus  in  the  brain 
it  may  be:  cerebral  proper,  cerebellar,  pontine,  bulbar,  basal.  In  the  cord 
it  may  be:  cervical,  dorsal,  lumbar,  sacral,  or  else  according  to  localization 
on  the  transverse  section  it  may  be:  posterior  tract  form  (ataxic),  postero- 
lateral form  (spastic  ataxic),  lateral  tract  form  (spastic),  poliomyelitic 
form;  syringomyelitic  form. 

Oppenheim  has  called  special  attention  to  several  uncommon  types, 
viz.  cervical,  sacral,  pseudo-tabetic  and  ocular. 

Cervical. — It  is  characterized  by  ataxia  of  the  upper  limbs,  anaesthesia 
in  hands  and  fingers,  astereognosis.  Sense  of  touch  alone  is  involved, 
but  not  pain  and  temperature.  The  tendon  reflexes  of  arms  cannot  be 
elicited.  In  the  lower  limbs  spastic  phenomena  are  present.  Patho- 
logically there  is  multiple  sclerosis  in  the  posterior  columns. 

Sacral. — Symptoms  of  bladder  and  rectum  are  prominent.  Sensory 
symptoms  are  present  in  the  anogenital  region.  Achilles  and  anal  reflexes 
are  absent.     The  disease  runs  in  remissions. 

Pseudo-tabetic. — It  presents  the  symptoms  of  tabes  except  Argyll- 
Robertson  pupil  and  optic  nerve  changes  which  are  different  from  those 
in  tabes.     The  disease  runs  an  acute  course  unlike  tabes. 

In  each  of  these  three  forms  eventually  in  the  course  of  the  disease 
other  symptoms  of  the  main  affection  will  make  gradually  their  appear- 


300  DISSEMINATED    SCLEROSIS 

ance,  but  the  symptoms  just  enumerated  will  throughout  remain  most 
conspicuous. 

In  the  ocular  form  the  disease  may  begin  in  and  remain  restricted  to 
the  optic  nerves  for  years  before  other  symptoms  will  begin  to  make  their 
appearance.     Involvement  of  the  ocular  motor  nerves  may  be  associated. 

In  the  cerebral  form  the  predominating  symptom  is  hemiplegia.  The 
latter  may  develop  gradually,  usually  with  remissions  and  exacerbations, 
or  by  apoplectic  seizures.  Hemiplegia  following  apoplexy  usually  dis- 
appears partially  or  even  totally  but  has  a  tendency  to  recur:  after 
several  attacks  it  may  remain  permanent. 

In  the  basal  or  ponto-bulbar  form  the  conspicuous  symptom  will  be 
crossed  paralysis.  In  the  cerebellar  form,  titubation,  nystagmus, 
asynergia  are  the  most  prominent  symptoms. 

Course,  Termination,  Prognosis, — The  course  of  the  disease  is  very 
irregular.  It  is  usually  chronic  and  progressive,  but  there  may  be  station- 
ary periods  and  even  amelioration.  Then  again  some  external  influence, 
as  a  cold,  excesses,  exertion,  traumatism,  an  intercurrent  infectious  dis- 
ease, may  aggravate  the  symptoms.  At  the  onset  there  may  be  either 
exclusively  cerebral  symptoms  (vertigo,  headache,  apoplectic  attack) 
or  only  spinal  symptoms,  especially  spastic  paraplegia,  or  else  visual 
symptoms. 

Prolonged  amelioration  is  rare  and  recovery  is  still  rarer.  The  disease 
may  last  from  ten  to  twenty  years,  but  also  less  than  two  years.  Death 
may  result  from  bulbar  paralysis  or  from  some  intercurrent  disease  such 
as  pneumonia. 

Diagnosis. — Tremor  from,  mercurial  intoxication  is  similar  in  character 
to  that  of  multiple  sclerosis,  but  in  the  former  affection  the  tremor  may 
appear  spontaneously,  while  in  the  latter  only  upon  a  voluntary  act. 

Paralysis  Agitans  will  be  recognized  by  its  passive  tremor  which  usually 
disappears  upon  a  voluntary  act. 

Hysteria  sometimes  presents:  vertigo,  hemiplegia,  tremor  and  difficulty 
of  speech — all  symptoms  of  multiple  sclerosis.  Buzzard  says:  disseminated 
sclerosis  in  its  earlier  stages  is  of  all  organic  diseases  most  commonly 
mistaken  for  hysteria.  The  presence  of  sensory  disturbances  and  of  spe- 
cial stigmata  will  help  considerably  in  making  the  diagnosis  of  hysteria. 
Westphal  and  others  described  a  form  of  pseudo-sclerosis,  in  which  except 
nystagmus  all  other  symptoms  of  multiple  sclerosis  are  present  and  from 
which  patients  make  a  complete  recovery.  The  following  symptoms  are 
present:  speech-defect,  slowness  in  movements  of  the  eyes  and  facial  mus- 
cles, fixed  expression,  tremor,  plus  knee-jerks;  psychic  disturbances, 
apathy,  sometimes  delirium;  finally  apoplectiform  attacks.     The  patho- 


DISSEMINATED    SCLEROSIS  3OI 

genesis  of  the  disease  is  obscure.  Nothing  of  significance  could  be  found 
at  autopsies.  However,  in  StrumpelPs  cases  a  leathery  consistence  of  the 
brain  and  a  slight  degeneration  of  the  crossed  pyramidal  tract  in  the  cord 
were  found. 

In  cerebellar  tumors  there  may  be  titubation,  nystagmus  and  optic 
neuritis,  all  symptoms  of  multiple  sclerosis,  but  the  persistence  of  intense 
headache  and  vomiting  characteristic  of  tumors,  abscence  of  spastic 
paralysis,  absence  of  remissions  and  recurrences  will  promptly  decide  the 
diagnosis. 

In  Paresis  there  may  be  disorder  of  speech,  tremor  and  spastic  gait, 
but  the  character  of  these  disturbances  is  so  decidedly  different  from  those 
of  multiple  sclerosis  that  an  error  is  hardly  possible.  The  paretic  speech  is 
tremulous,  but  not  syllabic.  The  tremor  is  constant  and  independent  of 
voluntary  effort;  it  is  a  fibrillary  tremor  evident  chiefly  in  the  tongue  and 
lips.  Besides,  the  mental  symptoms  of  paresis  will  soon  help  to  arrive  at  a 
conclusion.  Positive  Wassermann  reaction  and  cytological  examination  of 
the  cerebro-spinal  fluid  will  render  further  aid  in  establishing  the  diagnosis 
of  paresis. 

The  hemiplegic  form  of  multiple  sclerosis  may  sometimes  be  confounded 
with  a  cerebral  hemiplegia  caused  by  a  hemorrhage  or  softening,  but  the 
tremor,  nystagmus  and  scanning  speech  are  absent  in  the  latter.  More- 
over in  disseminated  sclerosis  the  apoplectiform  attacks  are  usually 
transient  and  prodromal  symptoms  absent. 

Etiology. — Traumatism  is  considered  as  one  of  the  causes.  I  have 
personally  observed  several  cases  in  which  the  symptoms  began  to  develop 
shortly  after  a  severe  trauma.  Oppenheim  observed  the  disease  after 
intoxications  with  metals  and  carbonic  dioxide.  P.  Marie  considers  in- 
fectious diseases  as  a  frequent  cause.  Striimpell  believes  that  multiple 
sclerosis  is  a  congenital  disease.  Syphilis  (congenital)  is  also  considered 
by  some  as  a  cause.  A  neuropathic  heredity  has  been  traced  in  some  cases. 
The  affection  appears  usually  at  the  age  of  from  twenty-five  to  forty-five 
and  rarely  in   children. 

Treatment. — Rest,  nutritious  food,  avoidance  of  exertion  and  inter- 
nally iodides,  arsenic,  silver,  cod-liver  oil,  may  be  tried,  but  little  can  be  ex- 
pected from  medications.  If  syphilis  is  suspected,  salvarsan  and  mercu- 
rials may  be  tried.  Electricity  should  be  avoided,  for  fear  of  increasing 
the  spasticity.  For  the  latter  warm  baths,  massage  and  passive  movements 
are  advisable.  For  treatment  of  apoplectiform  seizures  see  "Apoplexy." 
Thiosinamin,  fibrolysin,  tiodine,  which  is  thiosinamin-ethyl -iodide,  have 
been  used  for  absorption  of  sclerotic  tissue.  The  last  particularly  has  been 
tried  in  disseminated  sclerosis.     W.  Murrell  {Med.  Press  and  Circular,  1909) 


302  TRAUMA   OF    THE   CORD 

obtained  very  favorable  results.  In  his  cases  nystagmus,  speech,  ataxia 
have  all  improved  greatly.  The  drug  is  administered  hypodermically  in 
0.20  to  i  c.c.  Injections  are  made  daily.  In  one  of  his  cases  Murrell 
used  134  injections.  Local  swellings  with  rise  of  temperature  are  ob- 
served but  they  disappear  soon. 

X-ray  treatment  has  given  satisfactory  results  to  Raymond,  Babinski, 
Marinesco,  etc.  Tremor,  ataxia,  speech,  writing,  also  general  health,  all 
improved  from  repeated  seances  of  exposure  to  X-rays. 

SECONDARY  AFFECTIONS  OF  THE  SPINAL  CORD 

I.  Traumatic  lesions  of  the  cord  (concussion,  contusion,  sudden  com- 
pression) . 

II.  Slow  compression  of  the  cord  (tumors,  caries  of  the  vertebrae,  Potts' 
disease) . 

I.  Traumatic  Lesions  of  the  Cord 

A  trauma  of  the  spinal  column,  whether  it  is  a  fracture,  dislocation  or 
else  a  blow,  a  shock  of  any  sort  (a  fall  on  the  feet  or  on  the  buttocks) 
may  be  followed  by  an  injury  of  the  cord  itself.  The  latter  under  these 
circumstances  may  undergo  a  concussion,  a  contusion,  a  sudden  com- 
pression, laceration  or  a  complete  severance. 

A.  Concussion. — The  modern  investigations  tend  to  prove  that  mate- 
rial changes  may  occur  in  the  cord  in  so-called  concussion.  Rupture  of 
small  blood  vessels,  destruction  of  axis-cylinders,  some  rarefaction  in  the 
protoplasma  of  cells,  have  been  found  in  some  instances. 

Symptoms. — Immediately  after  the  accident  the  following  condition 
may  be  observed:  paralysis  of  all  or  only  the  lower  extremities;  the 
reflexes  are  lost,  the  sensations  are  diminished  and  the  sphincters  are 
disturbed.  Soon,  however,  all  these  symptoms  begin  to  improve  and 
gradually  the  patient  recovers  all  the  functions. 

The  chief  characteristic  of  concussion  is  its  transitory  nature.  But  if 
in  some  cases  the  above  symptoms  remain,  evidences  of  an  organic  lesion 
of  the  cord  are  then  present.  In  every  case  of  concussion  of  the  spinal 
cord,  hysteria  should  be  thought  of.  Charcot  had  shown  long  ago  that 
disturbances  following  railroad  accidents,  particularly  the  so-called  "rail- 
road spine,"  belong  to  traumatic  hysteria.  Stigmata  of  the  latter 
should  be  looked  for  in  such  cases. 

B.  Contusion. — It  presupposes  a  trauma  of  greater  severity  and  accord- 
ingly the  symptoms  are  more  pronounced.     It  is,  however,  difficult  to 


TEAUMA   OF   THE   CORD  303 

draw  a  sharp  line  between  concussion  and  contusion.  In  both  cases  some 
lesions  mentioned  above  have  been  found  in  the  cord  and  in  both  cases 
recovery  may  follow.  The  main  clinical  difference  between  the  two  lies 
in  the  longer  duration,  in  the  greater  severity  and  in  the  greater  possi- 
bility for  the  symptoms  to  remain  permanent. 

It  is  important  not  to  lose  sight  of  this  fact  that  an  accident,  whether  it 
is  a  concussion  or  contusion,  may  be  the  exciting  cause  for  development 
of  chronic  organic  diseases  of  the  cord,  provided  there  is  a  predisposition 
or  when  the  latter  preexisted  in  a  latent  stage.  The  injury  in  such  cases 
hastens  the  progress  of  the  dormant  preexisting  affections. 

C.  Sudden  Compression,  Laceration  and  Severance  of  the  Cord. — 
Fracture  and  dislocation  of  the  vertebrae  are  the  causative  factors  in 
this  condition. 

Pathology. — The  state  of  the  cord  is  various  according  to  the  intensity 
and  the  suddenness  of  the  injury.  Sometimes  the  cord  at  the  level  of 
the  traumatism  is  reduced  to  an  unrecognizable  soft  mass;  above  and  below 
that  level  the  nervous  tissue  gradually  undergoes  degenerative  changes: 
chromatolysis  of  cells  and  ascending  or  descending  secondary  degeneration 
of  tracts  of  fibers.  Should  a  secondary  infection  occur,  an  abscess  is 
likely  to  develop  in  the  cord. 

When  the  traumatism  of  the  cord  is  less  severe,  only  a  hemorrhage 
may  occur  either  in  the  cord  itself  or  in  the  membranes.  Rupture  of 
the  roots  or  of  the  spinal  ganglia  may  also  take  place.  The  secondary 
changes  above  and  below  the  injured  area  will  develop  like  in  the  first  case, 
but  to  a  less  extensive  degree.  It  should  be  borne  in  mind  that  in  injuries 
of  the  cervical  and  upper  dorsal  segments  the  cord  alone  may  be  injured, 
because  the  roots  at  those  levels  leave  the  cord  transversely.  In  injuries 
of  the  lower  dorsal  and  lumbar  segments  there  is  usually  a  simultaneous 
involvement  of  cord  and  roots,  for  the  latter  have  an  oblique  direction. 

Symptoms. — From  the  foregoing  remarks  it  can  be  seen  that  the  symp- 
tomatology will  vary  in  each  case.  In  injuries  of  the  cervical  and  upper 
dorsal  portions  of  the  cord  there  may  be  only  cord  symptoms,  while  in 
injuries  below  that  level  there  will  be  both  cord  and  root  symptoms. 
A  fracture  or  dislocation  of  the  vertebras  will  produce  a  deformity  of  the 
spine  which  can  be  recognized  by  the  usual  tests  for  surgical  diagnosis. 
It  must,  however,  be  borne  in  mind  that  no  deformity  may  be  observable 
when  a  fracture-dislocation  occurs  at  the  level  of  the  fifth  or  sixth  cervical 
vertebra.  The  same  may  occur  in  tuberculous,  non-suppurative  caries 
of  the  vertebrae  in  the  lumbar  region.  In  such  cases  skiagraphy  is  of 
great  aid.  It  can  accurately  determine  the  nature  and  the  seat  of  the 
trouble.     As  to  the  condition  of  the  nervous  system,  there  is  a  distinction 


304  TRAUMA    OF    THE    CORD 

to  be  made  between  a  simple  sudden  compression  or  a  severe  crushing 
of  a  segment  of  the  cord  produced  by  a  fragment  of  the  bone  or  by  a 
dislocated  bone.  In  both  cases  paralysis  will  be  the  most  conspicuous 
symptom.  When  the  cord  is  severely  injured,  the  paralysis  will  be  flaccid 
and  absolute  and  the  reflexes  totally  abolished.  In  simple  com- 
pression, on  the  contrary,  the  paralysis  is  spastic  and  the  reflexes  are 
increased. 

The  sensations  will  also  differ  in  both  cases.  In  severe  injuries, 
when  the  cord  is  totally  lacerated  or  crushed  at  the  point  of  the  injury, 
the  parts  below  the  lesion  are  totally  anaesthetic  to  all  forms  (touch,  pain 
and  temperature)  and  even  the  subjective  sensations  are  abolished. 
Immediately  above  the  anaesthetic  area  there  is  a  hyperaesthetic  zone 
which  is  due  to  an  irritation  caused  by  the  traumatic  focus.  In  incomplete 
injuries,  in  simple  compression  pain  will  be  present  in  the  limbs  and  follow 
the  course  of  the  nerves.  It  is  sharp,  lancinating  in  character.  The 
patient  will  also  complain  of  cold,  heat,  tingling  or  other  abnormal  sen- 
sations. The  objective  sensibility  is  also  disturbed:  there  is  a  diminution 
of  all  the  senses  or  sometimes  a  dissociation  (see  Syringomyelia).  In 
localizing  the  seat  of  the  lesion  it  should  be  borne  in  mind  that  the  upper 
border  of  the  anaesthetic  area  is  not  at  the  level  of  the  diseased  vertebra, 
but  it  is  decidedly  lower  (see  Anatomy  and  Fig.  92). 

The  sphincters  are  involved.     Retention  and  constipation  are  frequent. 

Trophic  disturbances  are  frequent.  Bed-sores  appear  rapidly  at  points 
of  pressure  (sacrum,  trochanter,  heels).  (Edema,  eruptions,  hydrarthrosis, 
muscular  atrophy,  are  not  infrequent  complications. 

Injuries  to  the  upper  cervical  region  deserve  special  mention.  Death 
may  be  sudden  (involvement  of  the  phrenic  nerve).  If  the  patient  sur- 
vives, there  may  be  paralysis  of  the  muscles  supplied  by  the  cervical  nerves, 
bulbar  symptoms,  radiating  pain  in  the  area  of  distribution  of  the  cervical 
nerves.  Erection  of  the  penis,  which  may  be  persistent,  is  a  common 
symptom  in  injuries  at  this  level  of  the  cord. 

In  injuries  of  the  lower  cervical  and  upper  dorsal  segments  of  the  cord, 
besides  the  symptoms  described  in  the  general  symptomatology,  there  are 
special   ocular  signs,  viz.   myosis  and  narrowing  of  palpebral  fissures. 

Course,  Termination,  Prognosis. — If  the  cord  is  totally  destroyed  at 
the  level  of  the  injury,  recovery  will  never  follow:  a  myelitis  will  be  the 
consequence,  and  the  patient  may  die  from  bed-sores  or  cystitis  with 
ascending  urinary  infection.  In  cases  of  mild  or  incomplete  compression, 
ascending  or  descending  degeneration  will  be  the  result  and  the  paralytic 
symptoms  are  permanent.  In  injuries  of  the  cervical  region  death  may 
be  instantaneous.     Dislocations  bear  a  less  grave  prognosis  than  fractures. 


TRAUMA    OF    THE    CORD  305 

In  some  cases  after  prompt  surgical  intervention  recovery  had  been 
observed. 

Treatment — When  a  sudden  compression  of  the  cord  occurs,  the  patient 
must  be  handled  with  extreme  care,  as  any  additional  movement  is  likely 
to  increase  the  compression.  Surgical  intervention  (laminectomy)  is  the 
only  treatment  in  such  cases.  As  it  is  sometimes  difficult  to  tell  the 
degree  of  the  damage  done,  it  is  advisable  to  postpone  the  operation 
for  some  days  and  during  that  time  observe  the  paralytic  symptoms. 
When  there  is  no  tendency  to  improvement,  a  prompt  operation  is  indi- 
cated. No  operation  is  necessary  in  cases  of  progressive  improvement. 
In  cases  of  an  evident  fracture  of  vertebrae,  a  prompt  intervention  is  urgent. 

In  order  to  determine  the  exact  area  of  the  injury  in  the  cord  and  thus 
outline  the  level  at  which  an  operation  is  to  be  performed,  there  are  two 
means  at  our  command.  First,  an  exact  and  thorough  study  of  the  sen- 
sory and  motor  disturbances  (see  Anatomy,  Fig.  92).  Second,  an  X-ray 
examination  of  the  spine. 

Surgeons  are  divided  as  to  the  time  of  interference:  some  are  in  favor 
of  an  early  operation,  others  operate  not  before  a  few  weeks  have  elapsed 
after  the  injury.  I  have  seen  favorable  and  unfavorable  results  in  both 
cases.     The  following  rules,  however,  may  be  kept  in  mind  as  a  guide. 

Early  Intervention  is  indicated:  when  the  nervous  disturbances  may  be 
attributed  to  a  fracture  of  an  arch  of  the  vertebra;  when  skiagraphy 
shows  the  presence  of  the  bullet  (in  case  of  fire  arms)  in  the  spinal  canal; 
when  in  case  of  fracture  with  dislocation  a  bloodless  reduction  of  the 
dislocation  has  failed  and  the  patient  presents  signs  of  a  partial  lesion. 

Late  Intervention  is  indicated:  when  early  intervention  was  not  done 
and  the  nervous  symptoms  do  not  improve  spontaneously;  when  in  the 
course  of  a  partial  lesion  there  is  an  ulterior  aggravation  of  symptoms 
(compression  by  a  callus).  In  cases  of  total  lesion  an  operation  is  con- 
traindicated.  The  comparative  value  of  early  and  late  operations  can  be 
seen  from  Chipault's  statistics:  in  early  operations  he  obtained  6  per  cent, 
recoveries,  6  per  cent,  improvements,  8  per  cent,  with  no  change  and 
79  per  cent,  deaths.  In  late  operations  he  obtained  8  per  cent,  recoveries, 
27  per  cent,  improvements,  39  per  cent,  with  no  change  and  25  per  cent, 
deaths. 

Some  claim  that  hemorrhages  into  the  spinal  canal  are  not  an  indication 
for  laminectomy.  I  have  seen  favorable  results  following  prompt  opera- 
tions in  such  cases. 

As  to  the  irremediable  conditions,  such  as  paralysis,  spasticity  and 
sphincter  disturbances,  the  treatment  is  the  same  as  in  the  systemic 
diseases  of  the  cord  (see  those  chapters). 


306  COMPRESSION   OF   THE    CORD 

II.    Slow  Compression  of  the  Cord.    Tumors.    Caries  of 
Vertebras— Potts'  Disease 

Etiology. — The  cord  may  undergo  slow  compression  from  tumors 
or  from  deformed  and  displaced  vertebrae.  Tumors  may  originate  in  the 
cord  itself,  in  the  meninges,  in  the  perimeningeal  cellular  tissue  or  in  the 
spine.  Displacement  of  the  vertebras  occurs  frequently  in  caries  of  the 
bony  structure  of  the  spine — in  Potts'  disease.  Here  the  changes  in  the 
cord  are  due  not  only  to  the  direct  pressure  of  the  deformed  vertebrae, 
but  also  to  the  tubercular  pachymeningitis  created  by  the  vertebral  disease. 

Pathology,  Tumors. — (i)  Those  of  the  cord  itself  are  not  infrequent. 
Thus  Schlesinger  found  that  among  302  tumors  of  the  cord  125  were  within 
the  cord  substance.  According  to  Flatau  the  relative  frequency  of 
extra-  and  intramedullary  tumors  is  as  2.6  to  1.  Tubercle  is  the  most 
frequent  of  all.  Its  favorite  seat  is  in  the  enlargements  of  the  cord.  It 
frequently  coincides  either  with  tuberculosis  of  the  vertebrae  or  of  the 
lungs.  It  originates  from  the  blood  vessels.  It  presents  a  hard  mass 
which  may  undergo  softening  in  the  center.  Around  it  the  nervous  tissue, 
which  is  under  pressure,  proliferates  and  secondary  degeneration  develops. 
Gummata  are  not  very  rare.  Gliomatous  tumors  of  the  cord  have  been 
described  in  the  chapter  on  Syringomyelia.  (2)  Meningeal  tumors  are 
usually  benign  in  character.  They  originate  on  the  inner  surface  of  the 
dura.  The  most  frequent  is  sarcoma.  Other  tumors  are:  psammoma, 
myxoma,  fibroma,  syphiloma,  tuberculoma,  lipoma  and  echinococci. 
The  latter  two  develop  externally  to  the  dura.  (3)  In  the  perimeningeal 
cellular  tissue  in  addition  to  the  above  tumors  carcinoma  has  been  ob- 
served. (4)  Tumors  of  the  vertebras  are  carcinoma  and  sarcoma.  The 
first  is  usually  metastatic  (from  cancer  of  stomach  or  other  organs).  The 
second  originates  in  the  surrounding  tissue.  The  vertebrae  thus  affected 
are  soft;  the  spinal  column  is  bent  and  thus  compresses  the  roots. 
Hydatid  cysts  are  rarely  met  with  in  the  vertebrae  or  in  the  cord;  they 
originate  from  the  intra-  or  perispinal  tissue.  The  majority  are  formed 
in  the  posterior  mediastinum  or  in  perivertebral  muscles.  They  invade 
the  spinal  canal  through  the  intervertebral  foramina.  Exceptionally  the 
vertebrae  and  cord  remain  intact.  Usually  there  is  scoliosis  and  compres- 
sion of  the  cord  and  roots;  the  destruction  of  the  latter  is  very  severe. 
Early  laminectomy  is  urgent.  (5)  Tumors  may  also  originate  in  the  roots 
and  in  their  meningeal  coverings.  Multiple  fibromata  are  localized  in 
the  roots  (neurofibromatosis)  and  they  are  most  frequently  found  in  the 
nerves  of  the  cauda  equina.  The  posterior  roots  are  oftener  involved 
than  the  anterior  ones.     Solitary  fibromata  are  not  accompanied  by  diffuse 


COMPRESSION    OF    THE    CORD 


307 


neurofibromatosis;  their  point  of  departure  is  the  dura.  The  most 
frequent  seats  of  meningeal  tumors  are  the  dorsal  region  and  the  cauda 
equina. 

The  state  of  the  nervous  tissue  in  cases  of  slow  compression  by  a  tumor 
lying  externally  to  the  cord  is  as  follows:  The  cord  is  flattened  or  presents 
a  depression.  It  is  pale  or  else  congested.  At  the  level  of  compression 
the  myelin  is  broken  up,  the  axis-cylinder  disappears;  instead  of  it  neu- 
roglia proliferates  and  around  the  blood  vessels  connective  tissue  prolifer- 


Fig.  1 01. — Tubercular  Pachymeningitis.    A  Case  of  Tuberculoma  of  the  Cord. 

{Original.) 

ates;  the  affected  segment  is  therefore  in  a  state  of  sclerosis.  Above  and 
below  this  level  ascending  and  descending  degeneration  develops.  The 
cells  of  the  gray  matter  at  the  point  of  pressure  are  either  in  a  state  of 
atrophy  or  completely  absent.  The  meninges  are  thickened  and  adherent. 
Primary  tumors  of  the  cord  and  meninges,  even  the  most  malignant  ones, 
do  not  produce  metastasis.  When  they  originate  in  the  vertebrae,  meta- 
stases are  not  rare. 

In  Potts'  disease  the  destructive  tubercular  process  of  the  bone  (pus 
and  fungosities)  perforates  the  posterior  vertebral  ligament  and  reaches 
the  dura;  the  latter  becomes  inflamed  and  proliferates  (pachymeningitis), 
The  tubercular  masses  covering  the  dura  undergo  softening,  form  small 
purulent  collections  at  first,  then  later  constitute  a  large  abscess.  On 
the  other  hand  the  tubercular  masses  may  become  organized,  then  scle- 
rosed. The  thickened  dura  is  found  then  surrounded  by  a  fibrous  mass. 
Whatever  the  process  may  be,  the  roots  and  the  cord  undergo  compression. 
The  changes  in  the  cord  are  identical  with  those  described  above.     They 


308  COMPRESSION    OF   THE   CORD 

are  generally  localized  at  the  level  of  the  peridural  inflammation  with 
secondary  degenerations.  Inflammation  of  the  pia-arachnoid  is  excep- 
tional and  may  be  due  to  a  toxic  infiltration  along  the  roots.  The  involve- 
ment of  the  cord  is  explained  in  two  ways:  (i)  mechanical  action  upon  the 
cord,  roots,  vessels  and  lymphatics,  and  (2)  the  action  of  tuberculous 
toxins.  An  excellent  illustration  of  the  latter  possibility  is  found  in  one 
of  my  cases  (/.  of  Nerv.  and  Ment.  Dis.,  1904),  in  which  the  cord  was 
found  to  be  entirely  free  in  the  vertebral  canal  and  yet  most  extensive 
tubercular  changes  were  observed  in  the  cord.  A  primary  parenchyma- 
tous tubercular  myelitis  without  meningitis,  without  caseation  has  been 
observed  (see  chapter  on  Myelitis). 

The  cerebro-spinal  fluid  is  always  modified  in  all  cases  of  compression 
of  the  cord.  There  is  frequently  an  increase  of  albumen  without  an  increase 
of  cellular  elements.  Cells  of  the  nature  of  the  neoplasm  in  the  cerebro- 
spinal fluid  have  been  observed  in  the  course  of  secondary  cancer  of  the 
meninges. 

Symptoms. — A  compressing  body,  whatever  its  nature  may  be,  may 
affect  the  cord  and  the  roots  or  each  individually.  As  to  symptoms  of  bony 
involvement  (in  cases  of  tumors  of  the  vertebras),  they  are:  local  swelling, 
curvature,  oedema,  rise  of  local  temperature,  tenderness  of  the  vertebrae 
and  spontaneous  pain. 

Root  Symptoms. — Irritation  of  roots  and  spinal  nerves  produces  pain, 
which  is  unusually  intense  and  of  a  neuralgic  type;  it  is  continuous,  but 
presents  paroxysms  of  exacerbation  brought  on  upon  the  slightest  move- 
ment. As  pressure  is  at  work,  the  nerves  are  in  a  state  of  neuritis.  This 
is  the  reason  of  the  accompanying  trophic  (eruptions)  and  objective 
sensory  disturbances  (anaesthesia)  in  the  areas  of  their  distribution. 
Gradually  loss  of  power  and  atrophy  develop.  The  patient  then  presents 
a  painful  paraplegia.  The  latter  is  frequently  observed  in  carcinoma  of 
the  spinal  column.  When  the  motor  symptoms  become  pronounced, 
the  pain  usually  subsides.  The  seat  of  the  pain  is  of  an  important  localiz- 
ing value.  It  is  certain  that  the  pain  refers  to  the  seat  of  the  lesion  or 
below  it,  but  never  above  it. 

Cord  Symptoms. — They  consist  essentially  of  motor  and  sensory 
paralysis.  Gradually  but  progressively  a  loss  of  power  becomes  established 
in  that  portion  of  the  body  which  lies  below  the  lesion.  The  paralysis 
may  be  flaccid  at  the  beginning  and  become  spastic  later  or  be  flaccid 
or  else  spastic  through  the  entire  course  of  the  disease.  Spastic  paraplegia 
is  the  most  frequent.  The  reflexes  are  abolished  in  the  first  and  increased 
in  the  second  form.  In  the  latter  case  there  may  be  also:  ankle-clonus, 
Babinski's  sign,  Oppenheim's  and  paradoxical  reflexes.     The  objective 


COMPRESSION    OF   THE    CORD  309 

sensibility  is  usually  altered.  There  may  be  complete  anaesthesia  to  all 
forms  of  sensations  or  a  sensory  dissociation  of  syringomyelic  type;  finally 
there  may  only  be  a  diminution  of  sensations  (hypaesthesia) . 

Babinski  in  1899  and  191 1  (Rev.  Neur.)  called  attention  to  a  form  of 
spastic  paraplegia  without  degeneration  of  the  pyramidal  tract.  Clinically 
there  is  contracture  of  the  legs  in  flexion,  knee-jerks  are  not  exaggerated, 
but  the  cutaneous  reflexes  are  so  much  increased  that  stimulation  of  the 
skin  of  the  paralyzed  limb  produces  rapid  flexion  (defense  movement). 
Since  then  the  phenomenon  has  been  observed  in  Potts'  disease  and  it  is 
due  to  a  gradually  progressing  compression  myelitis  without  marked 
degenerative  changes.  Consequently  prompt  removal  of  this  compression 
may  restore  the  patient  to  health.  Sicard  and  Gutmann  have  recently 
reported  such  a  case  (Rev.  Neur.,  1912).  The  defense  reflex  may  be  of 
practical  value. 

The  sphincters  are  most  of  the  time  involved.  Trophic  disturbances 
are  not  infrequent.  Bed-sores  are  formed  in  the  points  of  pressure  of  the 
body  (sacrum,  gluteal  regions,  trochanter,  malleoli).  Muscular  atrophy 
is  frequent. 

The  general  symptoms  just  described  vary  according  to  the  segment 
of  the  cord  involved.     Moreover  certain  regions  present  special  symptoms. 

Compression  of  the  Cervical  Cord. — (a)  When  the  upper  segment 
is  involved,  pain  will  be  present  in  the  neck  and  shoulder,  which  at  the 
same  time  will  be  anaesthetic.  Paralysis  of  the  muscles  covering  this 
region  will  produce  an  inability  to  rotate,  flex  or  extend  the  head.  All 
four  extremities  are  in  a  state  of  spastic  paralysis,  the  upper  more  than 
the  lower.  The  sensations  of  the  limbs  are  also  involved.  The  special 
symptoms  are:  slow  pulse,  paralysis  of  the  phrenic  nerve  (hiccough, 
vomiting,  disturbance  of  respiration).  The  compression  is  at  the  level 
of  the  first  four  cervical  segments  which  correspond  to  the  first  three  cervi- 
cal vertebrae. 

(b)  When  the  lower  cervical  segment  is  involved  there  are:  pain  in 
the  arms,  paralysis  of  all  four  extremities,  muscular  atrophy,  objective 
sensory  disturbances.  The  latter  present  the  following  distribution. 
When  the  lesion  is  at  upper  portion  of  the  cervical  enlargement  the 
anaesthesia  is  at  the  level  of  the  base  of  the  neck.  When  the  lesion  is 
lower,  the  upper  border  of  the  anaesthesia  descends  to  the  upper  limbs  and 
is  distributed  in  longitudinal  bands  parallel  to  the  axis  of  the  limbs.  The 
special  symptoms  consist  of  myosis  and  narrowness  of  the  palpebral 
fissure.  When  the  compression  is  at  the  level  of  the  fifth,  sixth  and  seventh 
cervical  roots,  the  atrophy  will  affect  the  muscles  of  the  roots  of  the  upper 
limbs  (Erb's  type) :  deltoid,  biceps,  brachialis  anticus,  supinator  longus. 


3IO  COMPRESSION   OF    THE   CORD 

scapular  muscles.  When  the  eighth  cervical  and  the  first  dorsal  roots 
are  under  pressure,  the  atrophy  will  affect  the  muscles  of  the  hands 
(Klumpke's  type) . 

Compression  of  the  Dorsal  Cord. — The  pain  will  be  present  around  the 
trunk  and  the  upper  line  of  the  anaesthesia  will  determine  the  level  of  the 
compression.  The  paralysis  will  affect  the  muscles  of  the  trunk  below 
the  lesion  and  of  the  lower  extremities.  It  may  be  flaccid,  especially  in 
complete  destruction  of  the  cord,  or  spastic.  In  the  first  case  the  reflexes 
are  abolished,  in  the  second  exaggerated;  the  latter  may  be  accompanied  by 
ankle-clonus,  Babinski's  sign,  Oppenheim's  and  paradoxical  reflexes.  The 
sphincters  may  be  involved,  and  the  more  pronounced  the  more  it  ap- 
proaches the  lumbar  enlargement. 

Compression  of  the  Lumbar  Enlargement. — Pain  in  the  lumbar  region, 
in  the  abdomen  below  the  umbilicus  and  especially  along  the  crural  and 
sciatic  nerves;  flaccid  or  spastic  paralysis  with  lost  or  increased  reflexes 
respectively;  (the  paralysis  is  flaccid  in  cases  of  complete  interruption  of 
the  cord;  spastic  paralysis  is  in  the  incomplete  lesions);  constant  involve- 
ment of  the  sphincters;  anaesthesia  in  the  paralyzed  portions  of  the  body — 
these  are  the  symptoms  observed.  The  last  symptom  deserves  special 
mention.  The  upper  border  of  the  anaesthesia  does  not  correspond  to  the 
level  of  the  compression  because  of  the  oblique  direction  of  the  roots;  it 
is  therefore  higher  than  the  point  of  compression.  The  cremasteric 
reflex  is  lost.  Achilles  reflex  is  preserved.  Atrophy  is  not  observed  in 
slight  compression  but  marked  in  deep  compression. 

Compression  of  the  Sacral  Cord. — The  pain  is  chiefly  in  the  area  of 
distribution  of  the  sciatic  nerve.  Paralysis  affects  the  gluteal  muscles, 
those  of  the  posterior  aspect  of  the  thighs,  of  the  legs  and  of  the  feet. 
Deformities  of  the  feet  are  constant  and  they  depend  upon  the  groups  of 
muscles  the  mcst  affected  (equino varus,  or  valgus).  The  knee-jerk  which 
depends  upon  the  integrity  of  the  second,  third  and  fourth  lumbar  segments 
are  normal  or  increased.  The  Achilles'  tendon  reflex,  which  is  controlled 
by  the  fifth  lumbar  and  first  sacral  segments,  is  lost.  The  sphincters  are 
involved.  Anaesthesia  occupies  the  postero-external  aspect  of  the  thigh, 
leg,  foot,  the  gluteal  and  sacral  regions.  Muscular  atrophy  is  present  in 
advanced  cases. 

Compression  of  the  Conus  Medullaris  and  Cauda  Equina. — This 
condition  is  described  in  the  chapter  on  Diseases  of  the  Conus. 

Course,  Termination,  Prognosis. — The  disease  usually  develops 
slowly  and  progressively,  with  vague  painful  and  paretic  symptoms.  The 
clinical  aspect  will  vary  according  to  the  localization  of  the  tumor  or  of 
Pott's  lesion.     The  most  frequent  form  is  that  of  spastic  paraplegia,  but 


COMPRESSION   OF   THE    CORD  311 

the  disease  may  simulate  any  form  of  spinal  cord  lesion.  When  the  tumor 
is  of  syphilitic  nature,  marked  improvement  in  the  symptoms  is  observed, 
if  specific  treatment  is  energetically  administered.  In  Pott's  disease  the 
symptoms  of  caries  of  vertebrae  may  exist  long  before  cord  symptoms 
appear.  Sometimes  the  cord  symptoms  appear  long  before  deformity  is 
observed.  In  Pott's  disease  the  prognosis  is  more  favorable  when  the 
motor  symptoms  are  unaccompanied  by  marked  sensory  changes  than 
when  the  latter  are  present.  It  is  unfavorable  when  fLaccidity  of  the 
limbs  follows  spasticity. 

Death  may  ensue  from  bed-sores,  ascending  urinary  infection  cr  in  case 
of  a  malignant  tumor  (cancer)  from  generalization  of  the  latter;  finally 
from  some  intercurrent  affection.  In  Pott's  disease  the  patient  may  die 
from  extension  of  the  tubercular  process  to  the  lungs.  Recovery  may 
sometimes  follow  in  Pott's  disease,  the  abscess  discharging  externally. 

The  prognosis  depends  greatly  upon  the  segment  of  the  cord  involved. 
When  the  compression  is  in  the  cervical  region,  rapid  death  may  follow. 
Tumors  of  the  vertebra?  which  are  almost  always  malignant,  are  more 
serious  than  tumors  situated  in  the  spinal  canal.  Among  the  latter  the 
intra-dural  are  more  benign  and  longer  tolerated  than  the  extra-dural. 
The  prognosis  in  spinal  tumors  as  a  rule  is  very  serious  if  surgery  does  not 
intervene. 

Diagnosis. — In  its  early  period,  before  the  paralytic  symptoms  are 
marked,  the  diagnosis  is  quite  difficult.  The  pain  may  be  confounded 
with  simple  neuralgia  (intercostal  or  sciatic)  or  lumbago.  Charcot  said 
that  bilateral  pain  is  frequently  of  spinal  origin.  A  careful  examination 
of  the  spine  may  reveal  a  deformity  and  lead  to  the  diagnosis. 

In  cases  of  vertebral  cancer  and  Pott's  disease  a  deformity  of  the  spine 
is  almost  always  present.  The  first  is  mostly  met  with  in  old  age;  the  de- 
formity is  round,  not  angular;  the  pain  is  the  predominant  symptom  and 
cachexia  is  marked.  Cancer  of  the  spine  is  usually  secondary  to  cancer  of 
theviscera.  The  latter  is  observed  in  young  people;  the  deformity  is  angular; 
pain  is  present  on  pressure;  there  is  cutaneous  hyperaesthesia;  the  spine  is 
kept  rigid;  subjective  pain  is  but  slightly  marked;  tuberculosis  of  other 
organs  is  frequently  present.  In  some  cases  of  Potts'  disease  there  is  no 
deformity  of  the  spine;  the  latter  may  be  slightly  or  not  at  all  perceptible. 
Alquier  out  of  fifteen  autopsies  has  found  eight  where  involvement  of  the 
spine  could  not  be  ascertained.  In  such  cases  rigidity  of  the  spinal  column, 
localized  pain  and  spinal  hyperaesthesia  to  heat  will  help  to  recognize  the 
disease.  A  flaccid  monoplegia  or  a  band  of  anaesthesia  indicates  root  in- 
volvement, while  spastic  paraplegia  or  anaesthesia  below  the  lesion  indi- 
cates that  the  cord  is  being  compressed.     The  above  mentioned  contrac- 


312  COMPRESSION    OF   THE   CORD 

ture  in  flexion  with  the  defense  reflex  may  be  of  assistance  in  determining 
the  degree  of  involvement  of  the  cord. 

In  the  paralytic  period  the  disease  should  be  differentiated  from  tabes, 
myelitis,  multiple  sclerosis,  multiple  neuritis  and  hysteria.  In  Tabes 
the  ocular  symptoms  and  the  ataxia  will  decide  the  diagnosis. 

Myelitis  (diffuse)  develops  very  rapidly;  the  motor  symptoms  appear 
at  the  beginning,  pain  is  usually  absent.  In  the  transverse  form  the  onset 
is  usually  acute,  the  paralysis  is  marked  and  the  objective  sensory  disturb- 
ances are  early. 

In  Multiple  Sclerosis  the  absence  of  sensory  disturbances,  the  presence 
of  tremor,  nystagmus  and  the  special  speech  are  characteristic. 

In  Multiple  Neuritis  the  muscular  atrophy  appears  very  early;  the 
nerve  trunks  and  the  muscles  are  tender  on  pressure ;  there  is  no  spasticity 
and  there  is  usually  a  history  of  intoxication  (alcohol,  lead,  etc.). 

In  Hysteria  there  may  be  rigidity  of  the  spine  with  hyperesthesia,  but 
the  sphincters  are  intact.  There  is  no  muscular  atrophy.  The  sensory 
disturbances  are  marked.  Finally  the  hysterical  stigmata,  the  sudden 
onset  of  the  paralysis  after  an  emotion,  will  help  to  decide  the  question. 

Treatment. — In  Potts'  disease  absolute  rest  and  immobilization  of  the 
spine  with  orthopedic  appliances  are  of  great  benefit.  At  the  same  time 
general  hygienic  measures  with  abundant  nutritious  food,  also  adminis- 
tration of  iodides,  iron,  arsenic  and  phosphates  should  be  kept  up.  Great 
care  must  be  taken  of  the  patient's  skin,  as  bed-sores  are  easily  formed,  also 
of  the  bladder,  as  urinary  infection  is  frequently  the  cause  of  death.  If 
syphilis  is  suspected,  mercurials  with  iodides  should  be  used. 

As  to  surgical  means,  there  are  two  methods:  one  consists  of  breaking 
the  bones  and  putting  them  in  place  (redressement  of  Calot),  the  other  is 
laminectomy.  The  first  has  given  in  some  cases  satisfactory  results,  but 
the  majority  of  surgeons  report  disastrous  consequences.  A  modification 
of  this  method,  consisting  of  extension,  followed  immediately  by  fixation  of 
the  spinal  column,  has  given  better  results.  Laminectomy  has  been  also  suc- 
cessful in  some  isolated  cases.  In  case  of  abscess  of  vertebrae  which  can  be 
determined  from  gradually  increasing  spasticity  and  contracture  of  the  par- 
alyzed limbs,  also  from  the  fever,  the  abscess  must  be  exposed  as  promptly 
as  possible  by  laminectomy  or  by  a  postero -lateral  operation.  The 
latter  appears  to  give  more  satisfactory  results.  In  Arch.f.  Klin.  Chirurgie, 
1909,  Wassiliev  reports  brilliant  results  by  this  prccedure  and  he  cites  very 
unfavorable  results  from  laminectomy.  If  the  operation  is  performed 
very  early  when  pachymeningitis  and  cord  symptoms  have  not  yet  made 
their  appearance,  there  is  hope  for  curing  the  patient.  When  the  disease 
is  old  and  the  tuberculosis  of  the  vertebras  is  cured,  laminectomy  is  indi- 


COMPRESSION    OF   THE    CORD  ,         313 

cated.  Finally  the  existence  or  non-existence  of  the  defense  reflex  (see 
above)  may  assist  in  deciding  the  question  of  operative  interference. 

The  wisest  procedure  is  to  apply  first  the  non-opetative  treatment,  and 
if  there  is  no  improvement  in  the  paralytic  symptoms,  an  operation  should 
be  attempted.     Cases  of  spontaneous  recovery  have  also  been  reported. 

In  cases  of  compression  by  tumors  the  following  considerations  may 
serve  as  a  guide.  Cases  of  primary  and  isolated  spinal  tumors  without 
angular  deformity  and  without  flaccid  paralysis  necessitate  immediate 
intervention,  especially  when  there  are  only  root  symptoms.  It  is  better  to 
perform  a  large  laminectomy  over  four  or  five  vertebras  than  to  wait  for 
cord  symptoms  which  cannot  always  disappear.  Tumors  accompanied 
by  grave  deformities  of  the  spine,  tumors  that  show  rapidly  developing 
cord  symptoms,  present  a  grave  surgical  prognosis.  In  such  cases  an  opera- 
tion can  be  undertaken  only  when  an  exact  localizing  diagnosis  is  made.  In 
such  cases  the  operation  will  be  only  palliative.  In  cases  of  flaccd  para- 
plegia with  loss  of  reflexes  no  operation  should  be  performed.  As  to  the 
seat  of  operation,  the  latter  should  be  performed  in  cases  of  intra-dural 
and  extra-medullary  tumors  at  the  level  of  the  highest  nerve-root  involved. 
In  view  of  the  oblique  direction  of  the  roots  emanating  from  the  spinal 
cord  it  is  important  to  remember  that  in  an  adult  in  order  to  determine  the 
number  of  the  roots  at  the  level  of  a  certain  spinous  process  it  is  necessary 
to  add  to  the  number  of  the  corresponding  vertebra:  one  in  the  cervical 
region;  two  in  the  upper  dorsal  region;  three  in  the  lower  dorsal  region 
(sixth  to  eleventh  vertebrae) ;  the  lower  portion  of  the  eleventh  dorsal  and 
the  interspinous  space  immediately  below  correspond  to  the  last  three 
lumbar  pairs. 

The  spinous  process  of  the  twelfth  dorsal  and  the  space  immediately 
below  correspond  to  the  sacral  pairs. 

Below  the  second  lumbar  vertebra  the  cord  is  no  more  involved,  but 
the  cauda  equina  is  (see  Fig.  96). 

Cancerous  vertebrae  should  of  course  never  be  operated  upon.  The 
immediate  danger  of  operation  lies  in  shock  or  hemorrhage  or  infection. 
The  mortality  depends  much  on  the  seat  and  the  nature  of  the  tumor. 
In  the  cervical  region  shock  especially  is  to  be  feared  by  reason  of 
proximity  of  the  medulla.  In  the  dorso-lumbar  region  where  because 
of  the  frequency  of  diffuse  infiltration  of  the  meninges  and  roots  extirpa- 
tion of  tumors  is  very  laborious,  hemorrhages  are  frequent.  Moreover 
the  presence  of  vesico-rectal  trouble  and  of  extensive  anaesthesia  facilitates 
infection;  the  mortality  therefore  is  high.  In  the  dorsal  region  tumors 
are  usually  benign  and  strictly  limited;  their  removal  is  quite  easy.  The 
mortality  is  not  so  great  as  in  the  other  localizations.     Tumors  of  bony 


314         •  MUSCULAR   ATROPHIES 

origin  present  a  high  mortality,  hydatid  cysts  still  higher.  Almost 
complete  recovery  follows  removal  of  fibromata  or  sarcomata  of  the 
meninges.  Removal  of  tumors  of  cauda  equina  has  given  but  mediocre 
results.  Before  an  operation  is  decided  upon  it  is  advisable  to  try  for  a 
short  time  a  thorough  course  of  antisyphilitic  treatment.  Severe  pain 
in  non-operable  cases  must  be  controlled  by  opium. 

CHAPTER  XX 

MUSCULAR  ATROPHIES 
Classification 

The  chief  forms  of  muscular  atrophies  are: 

I.  Progressive  muscular  atrophy  of  spinal  origin  (Aran-Duchenne). 
la.  Progressive  muscular  atrophy  of  infants. 

II.  Progressive  muscular  dystrophy  (myopathy),  which  embraces  the 
following  subdivisions: 

(a)  Pseudo-hypertrophic  type  (Duchenne). 

(b)  Scapulo-humeral  or  juvenile  type  (Erb). 

(c)  Facio-scapulo-humeral  type  (Landouzy-Dejerine). 

III.  Primary  neuritic  atrophy     (Charcot-Marie-Hoffman-Tooth). 

IV.  Arthritic  muscular  atrophy. 

I.  Progressive  Muscular  Atrophy  of  Spinal  Origin  (Chronic  Anterior 

Poliomyelitis) 
(Aran-Duchenne's  Type) 

Pathology. — The  main  lesion  consists  of  a  gradual  degeneration, 
atrophy  and  disappearance  of  the  cells  of  the  anterior  cornua  in  the  spinal 
cord.  Under  the  microscope  the  cells  appear  small,  their  NissPs  bodies 
have  partly  or  totally  disappeared,  the  nuclei  and  nucleoli  are  either  on  one 
side  or  outside  of  the  cells,  the  yellow  pigment  is  increased  in  amount. 
The  number  of  the  cells  is  considerably  reduced.  The  prolongations  form- 
ing at  the  periphery  of  the  cord,  the  anterior  roots  suffer  secondarily:  they 
undergo  degeneration  and  atrophy  and  appear  therefore  slender. 

The  degeneration  of  the  roots  is  continued  into  the  peripheral  nerves, 
but  to  a  lesser  degree.  The  nerve-terminations  in  the  muscles  are  dis- 
tinctly degenerated.  The  muscular  tissue  also  undergoes  changes: 
granular  degeneration  transforms  the  fibers  into  indistinguishable  masses, 
which  are  gradually  absorbed;  atrophy  is  the  result.  The  disease  is 
therefore  an  affection  of  the  lower  motor  neurons.  In  cases  with  bulbar 
symptoms  the  nuclei  of  motor  nerves  are  involved.  The  eleventh  and 
twelfth  are  most  frequently  affected.  The  ninth,  tenth,  seventh  and 
motor  portion  of  the  fifth  may  also  be  involved. 


MUSCULAR   ATROPHIES 


3*5 


The  above  changes  in  the  gray  matter  are  accompanied  by  proliferated 
neuroglia  tissue.  The  blood  vessels  are  dilated  and  thickened.  A  pri- 
mary degeneration  of  the  nervous  elements  of  the  gray  matter  is  the 
essential  characteristic  of  the  disease. 

Symptoms. — The  onset  is  characteristic.  In  the  majority  of  cases 
the  small  muscles  of  the  hands  are  first  affected.  The  thenar  muscles, 
the  superficial  first  and  the  deep  next,  are  first  to  be  involved.  The  ball 
of  the  thumb  then  becomes  flattened.  The  other  small  muscles,  the 
lumbricales,  and  the  interossei  follow.  The  spaces  between  the  meta- 
carpal bones  are  deep,  the  prominences  of  the  bones  become  marked. 


Fig.  102.  Fig.  103. 

Progressive  Muscular  Atrophy,  Following  Infantile  Spinal  Paralysis. 

The  first  phalanges  are  in  extension,  while  the  other  two  are  in  flexion. 
The  hand  is  claw-like  (main  en  griff e).  Gradually  the  atrophy  spreads 
tojihe  muscles  of  the  hypothenar,  of  the  forearm,  arm,  shoulder,  neck, 
thorax  and  lower  extremities  (Figs.  102,  103,  104,  105,  106). 

On  the  forearm  the  flexors  are  affected  before  the  extensors,  the  exten- 
sors of  the  fingers  before  the  extensors  of  the  wrist.  On  the  arm  the  atro- 
phy of  the  deltoid  muscle  alters  the  round  contour  of  the  shoulder,  the 
head  of  the  humerus  becomes  evident.  Atrophy  of  the  scapular  muscles 
brings  out  prominently  the  borders  of  shoulder  blade;  atrophy  of  the 


316  MUSCULAR   ATROPHIES 

serratus  projects  the  posterior  border  of  the  scapula  like  a  wing  (Fig.  106). 
Atrophy  of  other  muscles  of  the  extremities  of  the  neck  and  abdomen  will 
also  change  the  contour  of  the  bones  and  produce  corresponding  functional 
disturbances.  The  muscles  that  frequently  escape  rapid  wasting  are: 
the  latissimus  dorsi,  triceps  of  arm,  highest  portion  of  trapezius,  sterno- 
mastoid. 

The  functional  disturbances  are  first  manifested  in  the  hands.  The 
patient's  attention  is  attracted  to  an  awkwardness  in  performing  fine  acts, 
such  as  writing,  buttoning.  When  the  atrophy  advances,  the  patient 
becomes  helpless  and  his  attitude  is  quite  characteristic  when  he  attempts 
to  do  anything  at  all:  he  turns,  tries  to  help  himself  with  the  entire  body 
in  an  effort  to  do  what  the  hands  alone  should  have  done.  When  the 
muscles  of  the  neck  are  affected,  the  head  cannot  be  held  erect.  Atrophy 
of  intercostal  muscles  interferes  with  respiration,  which  then  becomes 
exclusively  diaphragmatic.  Atrophy  of  the  muscles  of  the  lower  extremi- 
ties will  interfere  with  standing  and  walking. 

The  other  characteristic  signs  of  the  disease  are :  fibrillary  or  fascicular 
contractions  of  the  affected  muscles,  also  reactions  of  degeneration. 
The  first  can  be  observed  almost  continuously,  and  the  least  stroke 
intensifies  them.  They  are  probably  due  to  an  increased  irritability 
of  the  cells  of  the  anterior  cornua  in  the  cord. 

The  reflexes  are  usually  diminished  for  want  of  muscular  tissue.  There 
are  no  changes  in  sensations  or  in  the  sphincters. 

Course,  Termination,  Prognosis.' — The  description  as  given  above 
is  met  with  in  typical  cases,  but  the  onset  as  well  as  the  successive  stages 
of  the  disease  may  vary.  Although  it  is  slow  and  progressive,  neverthe- 
less it  may  be  arrested  in  its  development  at  a  certain  period  or  for  a  certain 
time.  It  may  last  then  many  years,  as  long  as  fifteen  or  twenty.  In 
other  cases  it  may  suddenly  assume  a  subacute  development  and  advance 
rapidly.  Usually  the  patient  dies  from  some  intercurrent  disease,  as 
pulmonary  tuberculosis.  The  disease  may  have  also  an  ascending  course 
and  involve  the  nuclei  of  the  medulla;  the  patient  will  die  then  from  bulbar 
palsy. 

The  disease  may  also  present  an  atypical  onset:  instead  of  beginning 
in  the  muscles  of  the  hands,  it  may  affect  first  the  muscles  of  the  arm  or 
of  any  other  portion  of  the  body.  The  atrophy  may  begin  in  the  lower 
extremities.  In  such  cases  the  anterior  tibial  muscles  are  first  affected 
and  the  atrophy  gradually  extends  to  the  muscles  of  the  thigh,  etc.  When 
the  disease  begins  in  the  medulla,  atrophy  of  the  tongue  appears  first: 
difficulty  of  moving  the  tongue  in  every  direction,  flatness  of  its  muscula- 
ture,  fibrillary   tremor,    difficulty  in   pronouncing  words   are   the   chief 


MUSCULAR   ATROPHIES 


3J7 


symptoms.  Other  muscles  follow:  orbicularis  oris,  muscles  of  the  soft 
palate  and  of  the  vocal  cords.  When  this  occurs,  the  speech  becomes 
more  and  more  indistinct,  the  voice  is  low,  coughing  is  difficult,  blowing 
with  the  lips  is  impossible.  When  the  muscles  of  mastication  (masseter, 
temporals  and  pterygoids)  become  affected,  the  patient  cannot  chew  his 
food  and  the  mouth  does  not  close,  swallowing  is  very  difficult,  food 
regurgitates  through  the  nose  and  there  is  danger  for  food  to  enter  the 
larynx;  saliva  dribbles  constantly  from  the  mouth.  When  the  nucleus  of 
the  pneumogastric  becomes  involved,  dyspnea  and 
rapid  heart  action  develop.  When  the  disease  begins 
in  the  medulla,  the  patient  cannot  live  long  and  dies 
before  atrophy  appears  in  the  limbs. 

The  prognosis  in  all  varieties  of  the  disease  irre- 
spective of  the  mode  of  onset  is  always  unfavorable. 

Diagnosis.— Aran-Duchenne's  disease  is  so  typ- 
ical in  its  onset,  in  its  course  and  its  symptomatology 
that  in  the  majority  of  cases  the  diagnosis  is  not 
difficult.  Thus  it  will  be  readily  differentiated  from 
muscular  atrophies  occurring  in  the  course  of  other 
organic  diseases,  as  myelitis,  tabes,  hemiplegia,  etc. 
From  myopathies  it  will  be  distinguished  by  the 
onset,  fibrillary  twitching  and  RD.  In  syringo- 
myelia the  sensory  disturbances  will  decide  the 
question. 

Multiple  Neuritis  will  be  recognized  by  the 
presence  of  pain  and  paralysis,  also  by  the  absence 
of  fibrillary  contractions. 

Hypertrophic  cervical  pachymeningitis  presents 
a  history  of  pain  and  the  reflexes  are  usually  in- 
creased. 

Arthritic  muscular  atrophy  affects  the  muscles  of  hands,  Arms  Shoul- 

.        •      .  .  ders,  Face  and  Legs. 

only  in  the  vicinity  of  a  joint  which  was  previously 

diseased. 

Etiology. — Very  little  is  known  of  the  causes  of  this  affection.  Exposure 
to  cold,  trauma,  exertion,  have  been  reported  as  usual  causes.  Two 
cases  came  recently  under  my  observation  in  one  of  which  carrying  daily 
large  cakes  of  ice  in  the  left  hand  for  a  period  of  three  months  was  appar- 
ently the  only  accountable  cause  for  the  beginning  of  the  atrophy  in  the 
same  hand.  The  other  patient  sustained  a  blow  with  an  iron  bar  over 
his  shoulder.  There  was  no  erosion  or  any  other  apparent  lesion.  Shortly 
afterwaids  he  noticed  that  he  could  not  thread  a  needle  and  had  difficulty 


Fig.  i  o 4. — Progres- 
sive Muscular  Atrophy 
Showing     Involvement 


3*8 


MUSCULAR   ATROPHIES 


in  buttoning  his  vest.  Atrophy  began  to  develop  in  the  most  character- 
istic manner. 

Infectious  diseases,  intoxications,  syphilis,  have  been  also  mentioned 
as  causative  factors.  An  attack  of  acute  anterior  poliomyelitis  in  child- 
hood may  become  later  in  life  the  point  of  departure  of  progressive 
muscular  atrophy  (Figs.  102,  103). 

The  disease  usually  develops  in  middle  lif e,  more  frequently  in  men  than 
in  women.  In  a  few  isolated  cases  it  has  been  observed  in  several  mem- 
bers of  the  same  family  (Ormerod,  Holmes,  Gee). 


Fig.  105. — Progressive  Muscular  Atro- 
phy Confined  to  Upper  Arms,  Shoulders 
and  Thorax. 


Fig.  ic6. — Atrophy  of  the  Muscles  of 
the  Scapula  (Same  individual  as  Fig.  105). 


Treatment. — Massage  and  electricity  (especially  galvanism)  are  prac- 
tically the  only  means  of  treatment.  They  may  prevent  rapid  wasting. 
General  hygiene,  nutritious  food  and  avoidance  of  stimulants  are  indis- 
pensable. Mercuiials  and  iodides,  tonics — arsenic,  strychnine,  phosphorus, 
may  be  useful  in  some  cases. 

Progressive  Muscular  Atrophy  of  Infants. — Werdnig  (189 1)  and 
Hoffman  (1893)  described  a  variety  of  progressive  muscular  atrophy  in 
infants  which  pathologically  presents  the  picture  of  the  disease  just 
described,  but  clinically  differs  from  it.  The  characteristic  symptoms  are 
as  follows: 

In  the  midst  of  good  health  the  very  young  child  begins  to  lose  power. 
Soon  develops  a  distinct  paretic  condition  of  the  limbs  and  of  the  musculature 


AMYOTROPHIC   LATERAL    SCLEROSIS 


319 


of  the  trunk,  so  that  the  child  cannot  sit  up;  the  joints  show  a  looseness; 
the  muscular  atrophy  is  generalized  and  symmetrical;  there  is  some  adi- 
posity; the  reflexes  are  absent,  the  limbs  are  flaccid;  there  are  no  sensory 
disturbances,  no  involvement  of  the  sphincters  or  of  the  cranial  nerves. 
What  particularly  characterizes  this  affection  is  its  family  character  and 
its  short  duration,  viz.  from  two  to  three  years. 

The  disease  is  progressive  and  death  takes  place  from  some  intercurrent 
disease,  such  as  pneumonia,  etc.  The  pathogenesis  of  the  disease  lies 
probably  in  a  prenatal  disturbance  of  the  nutrition  of  the  cells  of  the  an- 
terior cornua  which  eventually  ends  in  their  destruction.  The  disease  is 
probably  allied  to  Oppenheim's  Myatonia  congenita. 

Amyotrophic  Lateral  Sclerosis 

(Charcot's  Disease) 

Pathology. — The  main  lesion  consists  of  atrophy  of  the  cells  of  the 
anterior  cornua  of  the  cord  and  a  sclerosis  of  the  pyramidal  tracts.  This 
double  lesion  is  found  frequently  in  the  corresponding  portions  of  the 


Fig.  107. — Amyotrophic  Lateral  Sclerosis.     (Original.) 

medulla,  pons  and  brain.  Thus  a  degeneration  of  the  pyramidal  tracts 
may  be  seen  besides  the  cord  in  the  subcortical  tissue  of  the  motor  area, 
in  the  internal  capsule,  in  the  pons,  in  the  medulla.  The  cells  of  the 
motor  nuclei  in  the  medulla  may  be  affected  at  the  time  as  the  cells  of 
anterior  cornua.     The  nucleus  of  the  hypoglossus  is  more  frequently 


320  AMYOTROPHIC   LATERAL    SCLEROSIS 

affected;  the  facial,  the  pneumogastric  and  the  motor  portion  of  the 
trigeminus  are  not  infrequently  involved.  The  large  pyramidal  cells  of 
the  cortex  of  the  motor  area  of  the  brain  may  be  equally  affected  in 
advanced  cases  (Fig.  107). 

The  changes  in  the  cord  are  more  pronounced  than  in  any  other 
portion  of  the  central  nervous  system.  In  view  of  the  changes  in  the 
cells  of  the  cornua,  their  prolongations,  anterior  roots  and  peripheral 
nerves  must  naturally  suffer.  Finally  the  muscles,  in  which  the  nerves 
terminate,  also  undergo  degeneration  and  atrophy. 

Taking  a  general  view  of  the  subject,  one  may  say  that  in  amyo- 
trophic lateral  sclerosis  two  systems  of  motor  neurones  are  affected,  viz. 
the  cortico-bulbo-spinal  and  the  peripheral  or  upper  and  lower.  The  first 
originates  in  the  large  pyramidal  cells  of  the  motor  cortex,  descends  through 
the  centrum  ovale,  internal  capsule,  crua,  pons  to  terminate  around  the 
motor  nuclei  of  the  medulla,  and  especially  around  the  ce]]s  of  the  anterior 
cornua  in  the  cord.  The  second  neurone  consists  of  the  cells  of  the  anterior 
cornua  (or  their  equivalents  in  the  medulla,  namely  the  cells  of  the  motor 
nuclei)  and  of  their  prolongations,  viz.,  anterior  roots  and  peripheral 
nerves. 

The  degeneration  of  upper  and  lower  motor  neurons  is  often  simul- 
taneous and  apparently  independent  of  each,  other.  The  cause  of  this 
primary  degeneration  is  still  unknown.  It  is,  however,  certain  that  old 
organic  diseases  of  the  central  nervous  system  may  start  the  pathologic, 
process.     Such  a  case  I  have  observed  (Amer.  Med.,  1903). 

As  to  the  histological  changes  of  the  cells  see  the  preceding  chapter  and 
of  the  fibers  see  the  chapter  on  Primary  Lateral  Sclerosis. 

Symptoms. — The  main  features  of  the  disease  are:  muscular  atrophy 
and  spasticity.  They  may  both  begin  simultaneously  or  one  follows  the 
other.  As  the  nuclei  of  the  medulla  may  also  be  affected,  there  will  be 
in  addition  to  the  above  symptoms  also  bulbar  disturbances. 

Cord  Symptoms. — The  lesion  of  the  cells  of  the  anterior  cornua  pro- 
duces muscular  atrophy  and  as  the  disease  is  chronic,  the  condition  in 
every  respect  is  that  of  Aran-Duchenne's  Muscular  Atrophy.  A  detailed 
description  of  it  is  given  in  the  preceding  chapter. 

The  lesion  of  the  pyramidal  tract  produces  spasticity.  The  latter  is 
particularly  observed  in  the  lower  extremities.  The  condition  is  that 
of  spastic  paraplegia.  The  gait  is  difficult  because  of  the  rigidity  and  the 
loss  of  power.  The  reflexes  are  increased.  Ankle-clonus,  Babinski's, 
Oppenheim's  and  paradoxical  reflexes  are  also  observed.  When  the  upper 
extremities  are  involved,  the  arms  are  adducted  to  the  trunk,  the  forearms 
pronated,  the  fingers  in  a  claw-like  position.     The  most  frequent  form  of 


MUSCULAR   ATROPHIES  32 1 

the  disease  is:  spasticity  with  weakness  in  the  lower  limbs  and  Aran- 
Duchenne's  atrophy  in  the  upper  limbs,  but  there  may  be  other  varieties 
according  to  the  portions  of  both  systems  of  neurons  most  markedly 
involved. 

Bulbar  Symptoms. — They  are  identical  to  the  labio-glossolaryngeal 
paralysis  of  Duchenne.  A  detailed  description  of  the  latter  was  given 
in  the  chapter  on  bulbar  paralysis,  in  which  its  relation  to  amyotrophic 
lateral  sclerosis  was  also  discussed.     (See  also  preceding  chapter.) 

Cerebral  Symptoms. — In  advanced  cases  in  which  the  cortical  cells 
and  the  subcortical  white  substance  are  involved,  mental  symptoms  are 
observed.  The  patient  is  emotional  and  mentally  feeble.  There  is  a 
tendency  to  laughing  and  crying. 

Course,  Termination,  Prognosis. — As  the  disease  may  affect  at  the 
beginning  each  system  of  neurones  (see  above)  separately,  the  onset  may 
be  various.  When  the  upper  extremities  are  first  affected,  the  muscular 
atrophy  will  be  the  initial  and  remain  as  the  most  conspicuous  symptom. 
When  the  lower  extremities  are  first  involved,  spastic  paraplegia  will  be 
the  most  noticeable  symptom. 

In  some  cases  the  disease  may  begin  in  the  medulla  and  remain  as 
the  most  prominent  symptom  for  a  long  time,  as  one  of  my  cases  shows 
{New  York  Med.  Jour.,  1907). 

The  disease  may  last  for  years,  if  the  bulbar  symptoms  are  late  to 
appear.     The  prognosis  is  invariably  bad. 

Diagnosis. — From  Progressive  Muscular  Atrophy  of  spinal  origin 
the  disease  will  be  differentiated  by  the  addition  of  spasticity  and  modi- 
fication of  the  reflexes.  Multiple  Sclerosis  is  recognized  by  its  typical 
speech,  nystagmus,  tremor. 

Amyotrophic  lateral  sclerosis  with  bulbar  onset  should  be  differentiated 
from  diseases  of  the  medulla  (see  this  chapter). 

Etiology. — Cold,  trauma,  exertion  are  mentioned  as  causes.  It  is  a 
disease  of  middle  life.  In  the  case  reported  by  me  the  boy  was  only 
fifteen,  and  the  disease  was  far  advanced.     (Amer.  Med.,  1903.) 

Treatment. — It  is  only  palliative.  Proper  hygienic  and  dietetic 
measures,  and  avoidance  of  stimulants  should  never  be  neglected.  To 
relieve  the  spasticity,  gentle  massage  and  warm  baths  will  be  useful. 
Iodides  and  mercurials  may  be  tried.  Electricity  and  strychnia  must  be 
avoided. 

II.  Progressive  Muscular  Dystrophy.    Myopathies 

Pathology. — The  lesion  is  in  most  cases  confined  to  the  muscular 
tissue.     The  nervous  system  is  in  the  largest  majority  of  cases  intact. 


322  MUSCULAR    ATROPHIES 

In  some  cases  atrophic  (not  degenerated)  cells  were  found  in  the  anterior 
cornua  of  the  spinal  cord.  This  condition  may  be  secondary  to  the  muscu- 
lar changes.  The  peripheral  nerves,  also  some  fibers  in  anterior  roots, 
have  been  found  altered  in  a  few  cases.  The  muscles  are  pale  and  atro- 
phied; their  individual  fibers  are  reduced  in  size  and  number;  they  be- 
come irregular  and  the  striation  disappears.  The  connective  tissue  be- 
tween the  fibers  is  proliferated  to  a  considerable  extent  (hyperplasia) 


Fig.  108.— Pseudo  Hypertrophic  Paralysis.     (Climbing  on  his  four.) 

and  contains  sometimes  a  large  amount  of  fat  cells.  In  some  cases  the 
muscular  fibers  instead  of  being  atrophied  are  hypertrophied.  In  this 
form  some  fibers  hypertrophy  at  first,  but  soon  they  atrophy  and  dis- 
appear, they  are  substituted  by  increased  connective  tissue  or  fat.  Atro- 
phic and  hypertrophic  varieties  depend  on  the  amount  of  connective 
tissue  or  fat  respectively.  The  disease  is  a  primary  muscular  affection 
independent  of  changes  in  the  cord  or  nerves. 

Symptoms. — (a)  First  Variety:  Pseudo-hypertrophic  Type. — It  oc- 
curs usually  in  young  children.  The  first  sign  of  the  oncoming  trouble 
is  noticed  in  walking.  The  child  falls  often  and  gets  tired  easily.  Upon 
examination  the  size  of  the  legs  is  found  to  be  out  of  proportion  with  their 


MUSCULAR   ATROPHIES  323 

function;  while  the  calf -muscles  are  enormous,  their  feebleness  is  strik- 
ing. On  palpation  the  muscles  are  either  firm  or  soft  according  to  whether 
connective  or  fat  tissue  respectively  predominates.  Of  other  muscles 
of  the  lower  extremities  the  extensor  of  the  knees  and  the  gluteus  maxi- 
mus  are  particularly  enlarged.  As  the  atrophy  spreads  to  the  pelvis  and 
abdomen,  the  patient  has  more  and  more  difficulty  in  walking;  in  the 
latter  act  the  pelvis  moves  markedly  up  and  down;  in  rising  from  a  seat 
the  patient  has  to  place  first  his  hands  on  his  thighs  and  help  himself  to 
get  up.  A  very  characteristic  sign  of  this  affection  is  the  manner  in  which 
the  patient  leaves  a  recumbent  position.  He  turns  to  either  side  with 
effort  in  his  first  attempt  to  sit  up;  then  he  tries  to  put  himself  in  a  kneel- 
ing position.  After  the  latter  is  reached,  he  places  his  hands  on  his  knees 
and  then  after  several  attempts  he  gradually  gets  up,  supporting  him- 
self continuously  with  his  hands,  which  slowly  slide  up  the  thighs  until  the 
erect  position  is  obtained.     (He  climbs  on  his  four,  Fig.  108.) 

The  atrophy  invades  gradually  the  upper  portions  of  the  body.  At 
this  time  lordosis  of  the  lumbar  spine  and  protrusion  of  the  abdomen 
develop.  The  patient's  gait  is  similar  to  that  of  a  duck.  As  the  disease 
is  essentially  progressive,  the  muscles  of  the  thorax  and  of  the  upper 
extremities  are  gradually  involved:  the  bones  become  prominent,  the 
shoulder-blades  move  abnormally  with  each  movement  of  the  arms.  The 
triceps  of  the  arm  is  usually  hypertrophied,  the  biceps  is  atrophied. 
The  muscles  of  the  hands  usually  escape. 

The  loss  of  power  has  this  characteristic  feature  that  it  develops 
simultaneously  and  in  proportion  with  the  muscular  wasting.  The  re- 
flexes decrease  also  parallel  with  the  degree  of  atrophy.  Fibrillary  con- 
tractions are  absent.  The  electrical  reactions  are  only  diminished  quan- 
titatively, but  there  is  no  RD.  Sensations  are  intact.  The  sphincters 
are  not  affected. 

(b)  Second  Variety:  Scapulo -humeral  or  Juvenile  Type  (Erb). — In 
this  form  the  myopathy  makes  its  first  appearance  in  the  muscles  of  the 
shoulder  and  upper  arm,  especially  trapezius,  serratus  magnus,  biceps 
and  triceps.  There  is  no  hypertrophy.  Gradually  the  atrophy  extends 
to  the  muscles  of  the  upper  and  lower  extremities,  also  of  thorax  and  pel- 
vis. The  disease  occurs  in  early  youth.  Electrical  reactions  are  only 
diminished  but  there  is  no  RD.     There  are  no  fibrillary  contractions. 

(c)  Third  Variety :  Facio-scapulo-humeralType  (Landouzy-Dejerine). 
— The  atrophy  affects  first  the  muscles  of  the  face  and  particularly  the 
orbicularis  oris.  In  a  well-developed  case  the  mouth  is  enlarged,  the 
occlusion  of  the  eyelids  is  incomplete;  the  facies  is  expressionless.  The 
patient  is  unable  to  blow,  whistle  and  pronounce  labial  letters. 


324 


MUSCULAR   ATROPHIES 


Gradually  the  myopathy  extends  to  the  shoulders  and  arms.  The 
rest  of  the  musculature  is  invaded  later  in  the  course  of  the  disease. 

The  affection  makes  its  first  appearance  in  adults.  Electrical  reac- 
tions are  only  diminished,  but  present  no  RD.  Fibrillary  contractions 
are  absent. 

Other  Varieties  of  Myopathy.— The  three  forms  of  myopathy  just 
described  received  their  names  from  the  mode  of  onset  or  from  the  pre- 
dominant seat  of  the  atrophy.  Observations  show  that  there  may  be 
other  varieties,  as  for  example  Zimmerlin's,  in  which  the  myopathy  com- 
mences in  the  upper  musculature  of  the  thorax  and  arm;  Eichorst's,  in 
which  the  femoro-tibial  muscles  are  first  affected.  It  is  useless  to  multiply 
the  names.  Goweis  in  1902  described  a  form  to  which  he  gave  the  name 
of  "Distal  Myopathy."  It  is  characterized  by  atrophy  confined  to  the 
muscles  below  the  elbow  and  the  knee. 

Course,  Termination,  Prognosis. — Irrespective  of  the  variations  in  its 
onset,  the  disease  is  essentially  progressive  in  character.  There  are  periods 
when  the  atrophy  arrives  at  a  certain  stage  of  development  and  remains 
stationary  for  a  certain  time,  but  finally  resumes  its  insidious  course.  As 
to  its  duration,  observations  show  that  the  earlier  in  life  the  atrophy 
begins,  the  shorter  is  its  duration.  The  pseudo-hypertrophic  patients  rarely 
reach  above  twenty.  The  ultimate  results  of  all  the  cases  are  absolute  loss 
of  power  and  confinement  to  bed.  Death  usually  results  from  some  inter- 
current disease  (pneumonia,  tuberculosis,  etc.) .  Life  is  in  danger  when  the 
respiratory  muscles  and  the  diaphragm  are  involved. 

Diagnosis. — Generally  speaking  there  is  no  special  difficulty  in  mak- 
ing a  diagnosis  of  myopathy  by  bearing  in  mind  the  essential  features 
described  above.  There  are,  however,  cases  which  present  some  obstacles 
in  arriving  at  a  positive  opinion  as  to  whether  they  belong  to  the  myopath- 
ics or  to  progressive  muscular  atrophy  of  spinal  origin.  The  fibrillary 
contractions  and  reactions  of  degeneration  which  used  to  be  considered  by 
the  elder  writers  as  pathognomonic  of  the  latter,  have  been  recently 
found  to  be  not  so  constant.  Moreover,  there  are  undoubtedly  cases  in 
which  some  groups  of  muscles  present  the  symptoms  of  one  form,  and 
other  groups  of  muscles  show  symptoms  of  the  other  form  of  muscular 
atrophy  (so-called  mixed  type) .  The  pathological  records  of  some  cases 
also  favor  this  view.  The  old  border  fine  can  no  more  be  considered  as 
sharply  defined  in  every  case.  There  is  a  certain  relation  and  affinity  as 
to  the  origin  between  various  forms  of  atrophy.  However,  for  practical 
purposes  it  is  a  good  plan  to  differentiate  them  according  to  the  special 
symptoms  described. 

Etiology. — Little  is  known  as  to  the  causes  of  this  affection,  except 


MUSCULAR    ATROPHIES  325 

that  it  is  met  not  infrequently  in  members  of  the  same  family.     It  is 
probably  due  to  some  congenital  defect. 

Treatment/ — Proper  hygienic  and  dietetic  measures  are  beneficial.  Lo- 
cally massage  and  electricity  are  indicated.  In  administering  the  latter 
it  should  be  borne  in  mind  that  muscles  undergoing  an  atrophic  process 
get  easily  fatigued.  Violent  electric  contractions  should  therefore  be 
avoided.  Galvanism  appears  to  have  a  better  effect  than  faradism.  As 
some  cases  of  this  affection  present  at  the  same  time  diminished  or  enlarged 
thyroid  glands,  administration  of  thyroid  extracts  or  perhaps  extracts  of 
other  ductless  glands  may  be  tried. 

m.  Primary  Neuritic  Atrophy  (Charcot-Marie -Hoffman-Tooth.)    Pero- 
neal Type  of  Progressive  Muscular  Atrophy 

Pathology.— The  lesion  consists  of  an  involvement  of  the  peripheral 
nerves,  of  the  spinal  ganglia,  of  the  posterior  roots  and  posterior  columns 
in  the  cord  and  of  the  cells  of  the  anterior  cornua.  The  most 
pronounced  changes  are  those  of  the  posterior  columns,  especially  of 
Brudach's.  The  latter  is  similar  to  that  of  tabes.  Hoffman  believed  that 
the  original  lesion  is  in  the  peripheral  nerves  (neuritis),  which  has  an 
ascending  course  and  eventually  involves  the  posterior  roots  and  the 
posterior  columns  of  the  cord.  Histologically  the  cells  are  in  a  state  of 
chromatolysis  and  finally  atrophy.  The  fibers  undergo  the  same  degenera- 
tive process  with  subsequent  sclerosis,  as  in  tabes.  The  peripheral  nerve- 
trunks  show  neuritis.     The  muscles  are  atrophied. 

Symptoms. — The  disease  begins  in  the  majority  of  cases  in  the  lower 
extremities,  and  especially  in  the  peroneal  group  of  muscles  (extensors) 
and  in  the  small  muscles  of  the  feet.  The  legs  appear  thin,  emaciated. 
The  contrast  between  the  size  of  the  legs,  especially  of  their  lower  thirds, 
and  the  rest  of  the  body  is  striking  and  quite  characteristic.  The  gait  is 
difficult  (steppage).  Foot-drop  is  evident.  Deformities  of  the  feet  are 
very  frequent  (pes  varus,  equinus  or  equino-varus) .  Gradually  the  small 
muscles  of  the  hands  and  of  the  forearms  are  invaded.  A  claw-like  hand 
develops.  The  atrophy  rarely  involves  the  proximal  ends  of  the  extremi- 
ties. It  may  extend  and  affect  the  muscles  of  the  trunk  and  of  the  face, 
but  this  is  rare  (Fig.  109). 

Fibrillary  contractions  are  observed  and  reactions  of  degeneration 
are  sometimes  present.  Diminished  response  to  faradism  and  galvanism 
is  common. 

The  reflexes  are  usually  diminished  or  abolished. 

Sensory  disturbances  (objective)  are  rare.  Pain  and  parassthesia 
may  occur.     In  my  case  (see  above)  the  disease  began  with  excruciating 


326 


MUSCULAR   ATROPHIES 


pain  in  the  feet,  but  when  the  atrophy  became  pronounced,  it  gradually 
disappeared.     Trophic  disturbances  (ulcerations)  may  occur. 
The  sphincters  are  intact. 

Course,  Termination,  Prognosis. — It  develops  very  slowly  and  may 
last  an  indefinite  number  of  years.     Life  is  not  threatened.     Remissions 

occur.  Death  usually  occurs  from  some 
intercurrent  disease  (pulmonary  or  other) . 
Diagnosis. — In  multiple  neuritis  the 
extensors  of  the  legs  are  also  frequently 
involved,  but  the  sudden  or  rapid  onset, 
the  paralysis  preceding  the  atrophy,  the 
tenderness  of  the  nerve- trunks,  the  eti- 
ology of  the  disease,  will  establish  the 
diagnosis. 

From  myopathies  the  disease  will  be 
differentiated  by  the  appearance  of  the 
atrophy  in  the5  distal  ends  of  the  limbs,  by 
the  fibrillary  contractions  and  RD. 

Dejerine  and  Sottas  have  recently 
described  a  disease  presenting  some  anal- 
ogy with  primary  neuritic  atrophy.  It 
is  called  "Interstitial  hypertrophic  neu- 
ritis." It  is  met  with  frequently  in 
several  members  of  the  same  family  and 
it  appears  early  in  life.  It  is  characterized 
by  a  muscular  atrophy  of  the  distal  ends 
of  the  limbs,  but  it  also  presents  a  tabetic 
symptom -group,  viz.  ataxia,  lancinating 
pain,  Argyll-Robertson  pupil.  There  is 
also  a  scoliosis  or  kyphosis.  The  periph- 
eral nerves  which  are  accessible  to  pal- 
pation are  markedly  thickened  (hypertrophied) .  Pathologically  it  pre- 
sents an  interstitial  neuritis  in  the  limbs,  also  a  certain  degree  of  degenera- 
tion in  the  posterior  columns  of  the  cord. 

Etiology. — The  disease  is  of  an  hereditary  character.  Several  members 
of  the  same  family  are  frequently  affected.  Men  suffer  more  than  women. 
It  is  a  disease  of  the  second  half  of  childhood.  In  one  case  reported  by 
me  (Jour.  Nerv.  and  Merit.  Dis.,  1903)  the  disease  developed  after  a  pro- 
longed exposure  to  cold. 

Treatment. — Massage  and  electricity,  orthopedic  appliances  and 
tenotomy,  are  all  the  means  we  have  at  our  command. 


Fig.  109. — Primary  Neuritic 
Atrophy. 


MUSCULAR   ATROPHIES  327 

IV.  Arthritic  Muscular  Atrophy. — It  accompanies  acute  and  chronic 
inflammations  of  the  joints.  It  affects  the  muscles  in  the  immediate 
vicinity  of  the  joints  and  rarely  extends  to  the  entire  limb  in  the  acute 
and  subacute  forms  of  rheumatism.  In  chronic  rheumatism  the  atrophy 
extends  to  the  muscles  lying  at  a  distance  from  the  diseased  joint.  In  the 
first  case  there  may  be  only  one  special  muscle  affected  or  else  the  group 
of  muscles  surrounding  the  joint.  In  the  second  case  the  atrophy  becomes 
diffused  and  involves  the  entire  limb.  Generally  speaking  the  degree  of 
atrophy  is  in  proportion  with  the  duration  of  the  arthritis  and  ankylosis 
of  the  joint.  When  several  joints  are  involved,  the  generalized  muscular 
atrophy  may  give  the  impression  of  Aran-Duchenne's  amyotrophy. 
The  differential  diagnosis  will  show  that  the  electrical  reactions  show  only 
a  quantitative  diminution,  but  no  reactions  of  degeneration.  Some 
authors  believe  that  the  inflammation  of  the  joint  spreads  to  the  neigh- 
boring nerves  and  the  atrophy  is  then  due  to  the  neuritis.  Others  think 
that  the  atrophy  is  caused  by  ischaemia  produced  by  the  swollen  joint. 
Others  attribute  the  atrophy  to  a  functional  inactivity. 

Finally  a  reflex  action  may  play  some  role.  There  are  some  path- 
ological evidences  that  the  sympathetic  system  may  be  the  underlying 
cause  of  arthritic  muscular  atrophy.  The  disease  tends  to  disappear 
when  the  joint  gets  well. 

Myatonia  Congenita  or  Amyotonia  Congenita 

In  1900  Oppenheim  called  attention  to  a  heretofore  unknown  con- 
dition which  is  characterized  by  a  generalized  muscular  weakness  with- 
out paralysis.  It  is  congenital  in  nature.  The  cranial  nerves  are  not 
involved.  The  intelligence  and  general  health  are  intact.  The  condition 
has  a  tendeny  to  improve. 

The  symptoms  are  noticeable  at  an  early  age,  viz.  from  birth.  When 
the  little  patient  is  seated,  the  trunk  bends  forward,  forming  a  marked 
kyphosis.  Placed  on  his  feet  he  is  not  only  unable  to  stand  alone,  but, 
even  when  supported,  his  legs  give  way  under  him.  That  there  is  no 
paralysis  can  be  seen  from  this  fact,  that  when  the  patient  is  on  his  back 
he  is  able  to  move  his  arms  and  legs.  The  weakness  (atonia)  affects 
also  the  ligaments  of  various  articulations,  so  that,  the  latter  can  be  placed 
in  hyperextension  and  in  all  possible  positions. 

The  muscles  are  soft  but  there  is  no  atrophy.  In  the  majority  of 
cases  the  electrical  reactions  of  the  muscles  are  normal.  In  grave  cases 
there  is  a  diminished  response  or  no  response  to  electrical  stimulation. 
The  skin  presents  a  certain  thickness,  reminding  one  of  a  myxedematous 


328 


MUSCULAR   ATROPHIES 


skin.  The  knee-jerks  are  usually  lost.  General  sensations  and  special 
senses  are  normal.     The  sphincters  are  not  involved. 

The  mentality  is  usually  intact.  The  muscular  atony  is  usually 
more  marked  in  the  lower  extremities,  but  it  may  equally  affect  the 
thorax,  neck  and  upper  extremities.  The  muscles  of  the  face,  tongue, 
eyes,  deglutition  are  not  involved.  The  growth  of  the  bones  appears 
to  be  normal. 

As  to  the  nature  of  the  condition  little  is  known.  The  two  autopsies 
placed  on  record  (Spiller,  Univers.  Penna.  Med.  Bull.,  1905,  and  Baudoin, 
Semaine  Med.,    1907)  reveal  nothing   characteristic  for  pathogenic    in- 


Fig.   no. — Myatonia  Congenita.     When  Sitting  Trunk  Falls  Forward. 

ferences.  However,  in  some  cases  an  increase  of  connective  tissue  and 
a  considerable  amount  of  fat  between  the  muscle  bundles  were  observed. 
Oppenheim  believes  that  the  disease  is  due  to  an  arrest  of  development 
of  the  muscle-fibers.  Some  writers  are  inclined  to  believe  in  a  disturb- 
ance of  function  of  some  ductless  gland.  Berti,  among  them,  considers 
amyotonia  congenita  as  a  variety  of  congenital  myxcedema.  While 
Amyotonia  congenita  presents  several  features  distinct  from  Myopathies, 
viz.  the  time  of  life  at  which  it  appears,  the  general  involvement  of  all 
the  muscles,  absence  of  deep  reflexes,  the  tendency  to  improvement, 
nevertheless  the  pathological  findings  which  show  that  there  is  no  essential 
difference  in  the  two  conditions,  the  not  very  infrequent  occurrence  of 
the  same  symptoms  in  both  maladies— all  these  facts  tend  to  show  that 
Amyotonia  congenita  probably  belongs  to  the  great  group  of  Myopathy ,[in 
which  so  many  variations  from  the  typical  forms  really  exist. 


SPINAL   MENINGITIS  329 

The  treatment  consists  of  good  hygienic  and  dietetic  measures  with 
massage  and  electricity.  A  natural  tendency  for  improvement  exists 
in  this  affection.     In  my  two  cases  the  improvement  was  remarkable. 

Myotonia  Atrophica 

Rossolimo  (Nouv.  Iconogr.  de  la  Salpetr.,  1902)  gave  this  name  to  an 
affection  which  was  described  before  as  Thomsen's  disease  with  muscular 
atrophy  by  Noques,  Tirol  and  Hoffman.  It  is  characterized  by  mus- 
cular atrophy  and  a  slow  relaxation  of  muscles  after  voluntary  con- 
traction. The  atrophy  is  characterized  by  its  distribution,  viz.  it  affects 
the  muscles  of  the  face  (myopathic  face) ,  the  sterno-mastoid,  the  vasti 
of  the  thighs  and  the  extensors  of  the  feet.  The  extensors  and  flexors 
of  the  forearm,  the  masseter  and  temporal  muscles  are  sometimes  affected. 
As  to  the  relaxation  of  muscles  after  contraction  the  stronger  the 
latter  the  longer  is  the  former.  The  electrical  reactions  show  a  dimin- 
ished response  to  faradism.  The  knee-jerks  are  absent.  Several  members 
of  the  same  family  have  been  found  to  be  affected,  males  more  frequently 
than  females.  It  appears  usually  at  the  age  between  fifteen  and  thirty, 
although  Grund  has  recently  reported  cases  of  myotonia  atrophica  at 
the  age  of  47  (Munch.  Mediz.  Wchnschr.,  April,  1913.)  The  disease  is 
essentially  progressive.  Pathologically  it  resembles  Mycpathy  as  far 
as  the  muscles  are  concerned.  Steinert  found  also  degeneration  of  the 
posterior  columns  in  the  cord.  Myotonia  atrophica  is  probably  one  of 
the  multiple  varieties  of  Myopathy. 

DISEASES  OF  SPINAL  MENINGES 

Spinal  Meningitis. — There  are  three  forms  of  this  affection: 

I.  Acute  spinal  meningitis. 
II.  Chronic  spinal  meningitis. 
III.  Hypertrophic  cervical  pachymeningitis. 

I.  Acute  Spinal  Meningitis 

This  form  of  meningitis  is  usually  a  secondary  affection.  In  the 
majority  of  cases  it  accompanies  an  acute  cerebral  meningitis.  In  some 
cases  it  may  be  primary  and  strictly  localized  to  the  membranes  of  the 
cord. 

Pathology. — An  incision  of  the  membranes  will  reveal  the  presence 
of  a  serous  or  purulent  exudate  and  adhesions  between  the  pia,  arach- 
noid and  dura.  The  membranes  are  hyperemic.  This  condition  may 
be  localized  cr  else  extend  over  the  entire  cord.  The  exudate  is  particu- 
larly marked  on  the  posterior  aspect  of  the  cord  and  it  covers  also  the 
cord  and  the  roots.  In  the  cerebro -spinal  form  the  lesion  is  confined 
only  to  the  pia  and  arachnoid.     In  the  tubercular  form  there  may  be 


33°  SPINAL   MENINGITIS 

either  isolated  tubercular  nodules  or  else  infiltrations  in  the  pia  around 
the  blood  vessels.  When  the  meningitis  is  secondary  to  a  disease  of  the 
vertebrae  (caries),  the  lesion  may  be  confined  to  the  dura,  but  it  may 
also  extend  to  the  pia;  purulent  formation  is  found  then  between  the 
spine  and  the  dura.  The  periphery  of  the  cord  is  always  in  a  state  of 
myelitis,  more  so  than  the  cortical  tissue  in  cases  of  cerebral  meningitis. 

Symptoms. — The  onset  is  sudden.  General  malaise,  fever  and  chills 
are  the  first  signs.  Pain  along  the  spine  is  the  most  prominent  symp- 
tom. The  latter  is  extremely  tender  to  touch  and  the  patient  immobil- 
izes his  spine  for  fear  the  least  movement  will  bring  on  pain.  The  pain 
radiates  around  the  thorax  and  to  the  limbs,  also  to  the  pharynx  and 
larynx.  It  is  continuous,  but  it  presents  also  paroxysms  of  exacerbation. 
The  entire  surface  of  the  body  is  hypersesthetic.  The  neck  and  back 
are  rigid.  Kernig's  sign  is  present.  It  consists  of  an  inability  to  ex- 
tend the  legs  when  the  patient  is  in  a  sitting  position.  The  reflexes  are 
increased.  The  sphincters  are  involved.  Vaso-motor  disturbances  are 
frequently  observed,  viz.  erythemata  and  sweating.  In  cervical  menin- 
gitis the  following  important  symptoms  are  observed:  vaso-motor  dis- 
turbances of  the  face,  pupillary  disturbances,  vomiting,  dyspnoea, 
dysphagia . 

If  the  patient  does  not  die,  he  enters  into  a  phase  of  paralysis,  which 
is  an  indication  that  the  spinal  cord  became  involved  (meningo-my elitis) . 
The  limbs  are  then  totally  paralyzed,  flaccidity  is  complete.  The  re- 
flexes are  lost,  anaesthesia  takes  the  place  of  hyperaesthesia  and  the 
sphincters  are  relaxed.     For  details  see  chapter  on  Myelitis. 

Course,  Prognosis.— While  death  may  occur  on  the  second  or  third 
day,  viz.  during  the  first  phase  of  the  disease,  it  may,  however,  be  pro- 
longed. In  the  majority  of  cases  the  termination  is  fatal,  but  recovery 
is  possible,  although  very  rare.  Death  is  due  to  the  involvement  of  the 
medulla.  In  recent  years  it  has  been  shown  through  the  study  of  cerebro- 
spinal fluid  that  there  are  benign  and  curable  (ambulatory  type)  forms 
of  meningitis  and  that  they  are  more  frequent  than  they  were  supposed 
to  be  in  former  years. 

Tubercular  meningitis  and  meningo-my  elitis  may  have  an  acute 
course,  but  as  a  rule  they  are  slow  and  progressive.  Infectious  menin- 
gitis, primary  or  secondary,  has  generally  a  rapid  course.  The  major- 
ity of  the  subacute  form  of  meningo-myelitis  are  either  tubercular  or 
syphilitic. 

Diagnosis. — From  acute  myelitis  the  disease  will  be  differentiated  by 
the  pain  which  precedes  the  paralysis.  The  latter,  together  with  anaes- 
thesia, are  much  later  symptoms  in  meningitis  than  in  myelitis. 


SPINAL   MENINGITIS  33 1 

Tetanus  will  be  recognized  by  trismus  and  paroxysms  of  contractions. 

Meningeal  hemorrhage,  which  also  produces  pain  and  rigidity,  is  not 
accompanied  by  fever. 

Etiology. — Infection  with  a  point  of  departure  in  the  neighboring 
tissues  is  the  chief  cause  of  acute  spinal  meningitis.  The  necessary  factor 
is  a  microorganism.  Streptococcus  and  pneumococcus  are  the  most 
frequent  ones.  Diseases  of  the  spine,  trauma,  bed-sores,  infectious 
diseases,  septicemia,  tuberculosis  and  syphilis,  compression  of  the  cord — 
are  all  causative  factors  in  meningitis. 

Treatment. — Applications  of  ice  to  the  spine,  cauterization  of  the 
latter,  hot  baths,  administration  of  iodides  and  mercurials  when  syphilis 
is  suspected,  hypnotics,  sedatives,  also  lumbar  puncture,  have  been 
advised  (see  also  Cerebral  Meningitis). 

II.  Chronic  Spinal    Meningitis 

It  is  a  common  secondary  lesion  in  various  diseases  of  the  cord. 
Occasionally  it  may  follow  an  acute  meningitis.  As  a  primary  affection 
it  is  found  in  senility,  in  lead  intoxication,  in  alcoholism.  Pathologically 
it  presents  thickened  membranes.  The  condition  of  the  meninges  second- 
ary to  cord  diseases  is  given  in  each  chapter.  The  symptoms  of  the 
primary  chronic  meningitis  are  analogous  to  those  of  the  acute  form  except 
that  they  are  less  marked  and  may  remain  latent.  There  is  pain  in  the 
back  and  in  the  neck.  The  rigidity  of  the  spine  is  but  slightly  marked. 
Fever  is  absent. 

As  a  special  form  of  chronic  meningitis  the  following  was  first  described 
by  Charcot  and  Joffroy: 

in.  Hypertrophic  Cervical  Pachymeningitis 

Pathology. — The  lesion  consists  of  a  thickening  of  the  dura  in  the 
cervical  region  of  the  cord.  The  thickened  inner  layer  of  the  dura 
adheres  to  the  pia  and  the  outer  to  the  endosteum  of  the  spinal  column. 
The  cord  surrounded  by  the  dense  fibrous  tissue  is  naturally  compressed. 
The  roots,  the  posterior  especially,  are  equally  compressed.  The  elements 
of  the  cord  and  the  nerve-fibers  of  the  roots  undergo  degeneration  and 
atrophy.  For  histological  details  of  the  latter  see  any  of  the  acute  or 
chronic  diseases  of  the  spinal  cord. 

Symptoms. — The  first  phenomenon  is  pain  in  the  neck.  It  is  con- 
tinuous and  presents  paroxysms  of  exacerbation.  It  radiates  to  the 
occipital    region,    shoulders    and    upper    extremities.     It    never    follows 


33 2  SPINAL   MENINGITIS 

the  course  of  nerve-trunks,  but  it  is  diffuse.  Sensations  of  tingling,  of 
numbness,  also  trophic  disturbances  (herpes)  are  also  present  in  the 
painful  areas.  The  entire  spinal  column  is  tender  and  the  neck  is  held 
rigid.  Objective  sensibility  is  altered,  hyperesthesia  is  present  at  first, 
but  later  when  the  nerves  degenerate,  anaesthesia  will  be  observed.  All 
these  phenomena  are  due  to  compression  of  the  posterior  roots. 

Gradually  (when  the  anterior  roots  become  involved)  the  painful 
phase  of  the  disease  gives  place  to  the  period  of  paralysis  and  atrophy. 
They  appear  first  in  the  upper  extremities.  The  atrophy  affects  mainly 
the  muscles  supplied  by  the  median  and  ulnar  nerves.  Flexion  and 
adduction  of  the  forearm  and  hand  are  impaired.  The  overextension  of 
the  wrist,  extension  of  the  basal  and  flexion  of  the  middle  and  last  phalan- 
ges gives  the  hand  a  special  position  (preacher's  hand)  that  is  characteristic 
of  the  disease.  The  atrophy  of  the  thenar  and  hypothenar  muscles  is 
marked.     RD.  is  present. 

Pain  usually  disappears  at  this  period  of  the  disease,  but  rigidity  of 
the  spine  persists.  Objective  sensory  disturbances,  hyperesthesia,  hypaes- 
thesia  or  anaesthesia  are  present  in  the  affected  areas.  In  a  more  ad- 
vanced stage  of  the  disease  (when  the  cord  itself  begins  to  suffer)  the 
lower  extremities  become  involved.  Spastic  paralysis  with  increased 
knee-jerks,  ankle-clonus,  Babinski's  sign,  Oppenheim's  and  paradoxical 
reflexes  will  be  present.  But  there  will  be  no  atrophy  in  the  paralyzed 
muscles.  At  this  phase  of  the  disease  the  sphincters  become  involved 
and  the  objective  sensory  disturbances  are  more  marked;  the  latter  are 
radicular,  viz.  they  follow  the  course  of  the  nerve- trunks.  Bed-sores  may 
also  develop.     The  cerebro-spinal  fluid  presents  lymphocytosis. 

Course,  Termination,  Prognosis. — The  disease  is  very  slow  in  develop- 
ment and  lasts  years.  The  painful  period  lasts  usually  several  months. 
Death  results  either  from  the  extension  of  the  morbid  process  to  the 
medulla  (bulbar  symptoms)  or  from  an  infection  caused  by  extensive 
bed-sores,  by  purulent  cystitis  or  else  from  an  intercurrent  disease  (pul- 
monary tuberculosis,  etc.).  Arrests  in  the  course  of  the  disease,  or  even 
considerable  amelioration  (perhaps  cure)  of  the  symptoms  have  been 
reported. 

Diagnosis. — Potts'  disease,  meningeal  tumors,  syringomyelia  may 
sometimes  be  confounded  with  hypertrophic  cervical  pachymeningitis. 
The  deformity  and  pain  produced  by  pressure  upon  the  cervical  spine  in 
Potts'  disease  are  characteristic.  Meningeal  or  vertebral  tumors  have 
their  special  signs  (see  these  chapters) .  The  diagnosis  with  syringo-myelia 
is  sometimes  difficult.  The  typical  sensory  dissociation  especially  will  en- 
able most  of  the  time  to  diagnose  syringomyelia. 


SPINAL   MENINGITIS  333 

Etiology. — Injuries,  prolonged  exposure  to  cold,  lead  intoxication, 
alcoholism,  syphilis,  are  all  considered  as  possible  causes  of  the  disease. 

As  a  primary  affection  hypertrophic  cervical  pachymeningitis  is  rare. 
Most  frequently  it  is  symptomatic  of  syringomyelia,  of  meningo-myelitis 
of  syphilitic  or  tubercular  nature.  It  may  participate  in  pachymeningitis 
of  the  base  of  the  brain. 

Treatment. — Local  counter-irritation  and  cauterization,  warm  baths, 
internal  administration  of  mercurials  and  iodides  may  be  tried.  Little 
can  be  expected  from  this  treatment.  Massage  and  electricity  may 
improve  the  muscular  atrophy. 

CIRCUMSCRIBED  SEROUS  SPINAL  MENINGES 

This  condition  of  the  meninges  has  been  described  only  recently.  It 
consists  of  a  circumscribed  accumulation  (pseudo-cyst)  of  cerebro-spinal 
fluid  in  the  sub-arachnoid  cellular  tissue.  It  is  probably  due  to  an  inflam- 
mation and  subsequent  adhesions  of  the  meninges  which  thus  produce 
an  increased  secretion  of  the  fluid  in  a  circumscribed  space.  This  pseudo- 
cyst is  found  in  the  dorso-lumbar  region  on  the  posterior  surface  of  the 
cord.  As  to  the  Etiology,  traumatism,  bacterial  infection,  a  toxic  process, 
are  considered  the  main  factors. 

Symptoms. — Horsley  insists  upon  unilateral  pain  at  the  onset.  Hyper- 
aesthesia  is  marked 

Anaesthesia  follows  the  hyperesthesia.  It  may  affect  all  forms  of 
general  sensibility  (touch,  pain  and  temperature).  Pressure  at  the 
level  of  the  roots  is  painful.  The  anaesthesia  may  vary  daily  as  far  as  its 
distribution  is  concerned.  Paraplegia  sets  in  insidiously,  first  in  one  leg 
and  then  in  the  other.  It  is  usually  spastic  but  flaccidity  may  follow 
spasticity.  The  reflexes  are  increased,  the  toe  phenomenon  is  present. 
Ankle-clonus  is  frequent.  Atrophy  may  be  present.  The  sphincters  may 
be  involved.     (Edema  has  been  observed. 

The  prognosis  depends  upon  the  early  recognition  of  the  affection 
and  upon  the  promptness  of  surgical  intervention.  If  abandoned, 
complete  paraplegia  with  its  usual  complications  (urinary  infection,  bed- 
sores, etc.)  will  develop.  Laminectomy  with  puncture  of  the  cyst,  followed 
by  washing  it  out  with  i  :  2000  of  bichloride  is  the  only  therapeutic 
procedure. 

HEMORRHAGES  OF  SPINAL  MENINGES 

They  may  occur  either  on  the  outer  or  inner  surface  of  the  dura. 
Extra-dural  Hemorrhages. — They  usually  occur  in  traumatism,  frac- 


334  INTERMITTENT    CLAUDICATION    OF    THE    SPINAL    CORD 

ture,  dislocation  of  the  spine,  penetrating  wounds,  caries  and  carcinoma  of 
the  vertebrae  and  meningeal  syphilis. 

Intra-dural  Hemorrhages. — The  preceding  factors  may  produce  this 
form  of  hemorrhage  but  less  frequently.  Infectious  diseases,  acute  and 
chronic  inflammation  of  the  meninges  (especially  syphilis),  instrumental 
deliveries,  hemorrhages  in  the  cranial  cavity— are  all  causes  of  spinal 
intra-dural  hemorrhages. 

Symptoms. — They  are:  pain  in  the  spine  radiating  to  the  limbs, 
rigidity  of  the  spinal  column,  paresthesia,  hyperesthesia.  Paralysis 
is  the  most  characteristic  symptom.  It  is  incomplete  at  first,  but  in 
two  to  three  days  becomes  complete.  Anaesthesia  is  also  slight  in  the 
beginning,  but  becomes  more  and  more  pronounced  later. 

The  sphincters  are  usually  involved.  The  knee-jerks  are  at  first 
diminished,  but  later  change  according  to  the  degree  of  compression. 

The  course  of  the  disease  depends  upon  the  amount  of  blood  in  the 
spinal  cavity.  Absorption  of  the  blood  may  take  place  in  slight  cases  and 
the  symptoms  then  gradually  disappear;  recovery  may  take  place  in  a  few 
weeks.  Frequently,  however,  complications  set  in:  secondary  meningitis, 
infection  from  bladder,  bed-sores,  etc.  The  prognosis  depends  upon 
the  seat  of  the  hemorrhage.  If  it  is  in  the  cervical  region,  death  may  be 
rapid  from  bulbar  symptoms.  If  hemorrhage  is  abundant,  cerebral 
anemia  and  syncope  may  follow.  Generally  speaking,  the  disease  lasts 
from  two  to  three  months  and  usually  leaves  motor,  sensory  and  sphincter 
disturbances. 

The  diagnosis  can  be  made  chiefly  from  lumbar  puncture. 

The  treatment  consists  of  absolute  rest  in  bed.  The  patient  should 
be  placed  on  his  side  in  order  to  avoid  congestion  of  the  meninges  and 
lungs.  Application  of  ice  and  counter-irritation  at  the  level  of  the 
suspected  hemorrhage  are  the  next  indication.  Pain  must  be  relieved 
by  the  usual  means.  Finally  repeated  lumbar  punctures  are  very 
important. 

INTERMITTENT  CLAUDICATION  OF  THE  SPINAL  CORD 

In  1906  (Reane  Neurologique)  Dejerine  called  attention  to  the  follow- 
ing symptom  group.  In  the  midst  of  apparently  good  health  the  patient 
notices  that  while  walking,  one  of  his  legs  gets  more  easily  tired  than  the 
other,  but  after  a  rest  this  fatigue  disappears.  For  a  long  time,  sometimes 
years,  he  will  complain  of  this  unilateral  weakness,  but  sooner  or  later 
the  other  leg  will  become  similarly  affected.  Gradually  the  interval 
after  which    the    weakness  of  the  limbs  appears,  becomes  shorter  and 


INTERMITTENT    CLAUDICATION    OF    THE    SPINAL    CORD  335 

shorter  and  a  typical  spastic  paraplegia  develops.  In  the  beginning  of 
the  intermittent  spinal  claudication  disturbances  of  the  sphincters  are 
observed,  either  retention  or  incontinence,  but  especially  imperative 
micturition,  frequently  also  sexual  impotence.  The  patellar  tendon 
and  Achilles  reflexes  are  exaggerated,  more  particularly  in  the  limb 
whose  function  is  disturbed.  Ankle-clonus  may  be  absent.  The  toe 
phenomenon  is  frequent.  The  terminal  spastic  paraplegia  may  take 
place  in  months  or  in  many  years.  The  latter  is  the  most  frequent  oc- 
currence. In  rare  cases  the  condition  may  run  an  acute  course,  viz. 
the  paraplegia  develops  shortly  after  the  onset  of  the  claudication. 

The  diagnosis  is  to  be  made  from  intermittent  claudication  of  peri- 
pheral origin.  In  the  latter  affection  the  condition  is  at  first  also  unilateral; 
the  walking  brings  on  rapidly  a  fatigue,  but  there  is  pain  which  is  frequently 
very  intense;  it  is  a  cramp  that  affects  the  entire  limb.  But  the  reflexes 
are  normal,  there  are  no  disturbances  of  the  sphincters.  On  the  other 
hand  we  find  here  the  limb  cold,  and  after  a  walk  it  becomes  cyanosed,  the 
cyanosis  disappears  after  a  rest.  The  most  important  sign  of  the  clau- 
dication of  peripheral  origin  is  the  disappearance  of  normal  pulsations 
in  the  dorsalis  pedis  artery  at  first  and  then  in  the  posterior  tibial  artery; 
sometimes  the  pulsation  cannot  be  felt  in  the  popliteal  and  femoral  arteries. 
Skiagraphy  often  shows  an  atheromatous  condition  of  these  arteries. 
Recoveries  may  occur  because  of  the  supplementary  collateral  circula- 
tion, but  most  frequently  the  condition  ends  in  gangrene  Its  etiology 
is  therefore  an  endarteritis  and  atheroma;  it  occurs  in  cases  of  alcohol- 
ism, gout  and  syphilis,  or  else  it  may  follow  an  acute  endarteritis  after 
typhoid  fever  in  young  people. 

A  different  condition  is  found  in  intermittent  spinal  claudication. 
Here  the  reflexes  are  exaggerated,  the  toe  phenomenon  is  frequent  and 
sphincter  disturbances  are  present.  Finally  the  arterial  pulsations  are 
normal  and  there  are  no  vasomotor  disturbances  in  the  affected  limbs;  the 
patient  feels  no  pain  while  walking  but  only  a  heaviness  in  the  limbs.  The 
Pathogenesis  of  the  affection  lies  in  a  very  slow  process  of  arteritis  which 
leads  to  a  narrowness,  of  the  lumen  of  the  arteries  supplying  the  segments 
of  the  dorso-lumbar  region  of  the  spinal  cord. 

As  to  the  etiology  of  the  affection,  infectious  disease,  such  as  grippe 
(case  of  Dejerine  and  Poix)  may  be  the  cause.  The  most  frequent 
etiological  factor  is  syphilis.  The  anti-specific  medications,  such  as 
Salvarsan,  mercury  and  iodides,  when  promptly  administered  may  im- 
prove the  condition  or  at  least  delay  for  a  very  long  time  the  oncoming 
terminal  Spastic  paraplegia. 


CHAPTER  XXI 

SYPHILIS  OF  THE  NERVOUS  SYSTEM 

The  microorganism  discovered  by  Schaudinn  and  Hoffmann  as  being 
the  cause  of  syphilitic  lesions  is  known  under  the  name  of  spirochasta 
pallida.  The  syphilitic  poison,  whatever  its  intimate  nature  may  be, 
appears  to  have  a  special  predilection  for  the  nervous  system.  Its  effect 
upon  the  latter  may  be  manifested  in  two  different  forms.  In  one  of 
them,  to  which  tabes  and  paresis  belong,  the  lesions  were  supposed  to  be 
until  recently  not  the  direct  and  immediate  result  of  syphilitic  infection, 
but  a  late  and  secondary  development  (degeneration),  against  which  the 
anti-syphilitic  treatment  is  powerless.  They  are  the  "parasyphilitic 
affections"  of  Founder. 

Our  former  view  concerning  the  relationship  of  syphilis  to  tabes  and 
paresis  must  now  be  modified.  Noguchi  and  Moore  (J.  of  Exper.  Med., 
Feb.  i,  1 913)  have  found  the  spirochaetse  in  the  brains  of  12  paretics, 
and  Noguchi  found  them  in  the  posterior  columns  of  a  tabetic.  In  the 
brain,  they  are  seen  more  numerous  in  the  cortical  than  in  the  white 
matter,  they  are  scattered  in  groups  between  the  cells  and  neuroglia 
fiber.  They  are  rare  in  the  vicinity  of  the  blood-vessels  and  not  found 
in  the  walls  of  the  latter.  These  findings  have  been  corroborated  by 
Levaditi,  Marie  and  Bankowsky  (Presse  Med.  No.  36,  1913).  The 
parasyphilitic  diseases  are  therefore  very  probably  true  syphilitic 
affections. 

The  other  form  with  which  we  will  be  exclusively  concerned  here  is 
characterized  by  distinctly  specific  lesions  due  to  the  direct  effect  of  syphi- 
lis. They  are  amenable  to  antisyphilitic  treatment,  especially  at  the 
beginning. 

Syphilis  may  affect  the  cerebro-spinal  system  at  any  period  of  its 
development.  Generally  speaking,  however,  cerebral  syphilis  occurs  in 
the  tertiary  period,  while  spinal  syphilis  is  an  early  occurrence,  viz.  in 
the  first  or  second  year,  even  as  early  as  in  a  few  months. 

In  a  syphilitic  individual  a  trauma,  excesses,  especially  of  alcohol,  and 
exhaustion  are  apt  to  be  the  exciting  causes  for  the  development  of  syphi- 
litic cerebro-spinal  disturbances. 

336 


SYPHILIS    OF    THE    NERVOUS    SYSTEM 


337 


SYPHILIS  OF  THE  BRAIN 

Pathology. — Meninges,  brain  tissue  and  blood  vessels  are  affected. 
In  the  meninges  the  specific  gummatous  formation,  which  is  the  char- 
acteristic lesion  of  syphilis,  may  be  diffuse  or  circumscribed.  Gummata 
may  be  of  various  sizes.  As  soon  as  they  are  formed,  they  become  a 
source  of  irritation  and  inflammation  to  the  neighboring  tissue.     They 


Fig.  hi. — Syphilitic  Meningitis  of  the  Bas       l   •  nE  Brain.     (Flatau,  Jacobson,  Minor.) 
On  the  left  are  seen  strong  adhesions  between  the  dura,  pia  and  underlying  cerebral  tissue. 

extend  into  the  latter  along  the  sheaths  of  the  blood  vessels  of  the  pia 
mater.  Adhesions  of  the  meninges  and  softening  of  the  nervous  tissue 
follow.  The  most  frequent  seat  of  gummata  is  the  base  of  the  brain 
from  the  chiasma  to  the  pons.  There  the  gumma  starts  from  the  pia  and 
rarely  involves  the  dura.  The  optic  and  oculomotor  nerves,  also  the  large 
basal  arteries,  especially  the  middle  and  anterior  cerebral,  are  usually 
involved.  When  situated  on  the  convexity,  they  are  usually  found  in 
the  frontal  or  parietal  regions.  The  obliteration  of  the  blood  vessels  will 
produce  softening  of  the  brain  tissue.     When  the  lesion  is  diffuse,  the 


338  SYPHILIS    OF    THE    NERVOUS    SYSTEM 

meninges  are  covered  with  a  thick  gelatinous  exsudate.  Histologically  a 
gumma  consists  of  a  granulation  tissue  formed  by  proliferation  of  connec- 
tive tissue  and  endothelial  cells.  It  is  very  vascular  and  presents  casea- 
tion in  some  places.  Round  cells  are  in  abundance  along  the  adventitia 
of  the  blood  vessels. 

Gummata  primarily  developed  in  the  brain  are  rare.  Diffuse  gum- 
matous infiltration  of  cerebral  tissue  may  also  occur.  It  may  affect  all  the 
three  membranes  or  only  the  pia-arachnoid.  The  dura  mater  when  in- 
volved becomes  very  thick  and  forms  adhesions  with  the  other  membranes 
and  brain  tissue  (gammatous  pachymeningitis.  The  cortex  is  usually 
involved,  the  lesion  will  be  then  a  syphilitic  meningo -encephalitis. 

The  cranial  nerves,  which  are  frequently  involved  in  basal  meningitis, 
may  also  be  affected  independently.  In  such  cases  there  is  a  round  cell 
infiltration  in  the  epineurium;  thick  processes  are  sent  out  between  the 
bundles  of  the  nerve-fibers.     The  latter  being  compressed  undergo  even- 


A 


Fig.  112. — Large  Gumma  in  the  Pons.     (Flatau,  Jacobson,  Minor.) 

tually  degeneration  or  atrophy.  The  condition  is  therefore  at  first  an 
interstitial  neuritis.  The  chiasma,  the  optic  and  the  oculo-motor  nerves 
are  most  frequently  affected.  The  fourth,  fifth,  sixth,  seventh  and  eighth 
are  next  in  frequency.  The  remaining  four  nerves  are  almost  never  involved. 
Syphilis  has  a  special  predilection  for  the  blood  vessels  of  the  base  of 
the  brain,  although  specific  arteries  may  be  encountered  in  any  portion  of 
the  brain.  The  lesion  consists  of  a  small  gummatous  formation  or  infil- 
tration in  the  walls  of  the  blood  vessels.  Microscopically  there  is  a  cell 
hyperplasia  in  the  lining  endothelium  of  the  vessel  and  the  fenestrated 
membrane.  The  effect  of  the  arterial  lesion  will  be  either  thrombosis 
or  rupture  of  the  vessel.  Softening  of  cerebral  tissue  or  hemorrhage 
will  be  the  consequence,  provided  collateral  circulation  is  not  established. 
Such  is  the  case  with  occlusion  of  the  terminal  arteries  in  the  internal 


SYPHILIS    OF    THE    NERVOUS    SYSTEM  339 

capsule,  basal  ganglia,  crura,  pons  and  medulla  and  the  nuclei  of  the 
cranial  nerves  from  the  third  to  the  twelfth  inclusive. 

In  congenital  syphilis  the  encephalitis  is  of  the  sclerotic  form,  rarely- 
softening. 

All  the  lesions  enumerated  may  be  observed  in  the  same  case.  In 
fact  multiplicity  of  lesions  is  frequent  and  characteristic  of  syphilis  of  the 
nervous  system. 

Symptoms. — The  foregoing  remarks  indicate  that  the  clinical  picture 
will  vary  with  the  localization  of  the  lesion.  A  syphilitic  basal  meningitis, 
a  diffuse  meningitis  of  the  cortex,  a  circumscribed  gumma  in  various 
portions  of  the  brain,  a  generalized  syphilitic  arteritis — all  these  forms 
naturally  have  their  special  symptoms.  Irrespective  of  the  form  or  of  a 
special  localization,  cerebral  syphilis  presents  a  prodromal  period  charac- 
terized by  the  following  symptoms. 

Headache  is  the  most  constant  and  the  earliest  phenomenon.  Its 
essential  feature  is  to  present  exacerbations,  especially  in  the  evening 
or  at  night,  and  to  be  deep  seated  in  some  part  of  the  cranium.  During 
the  day  it  is  dull  or  may  disappear  entirely.  The  headache  yields  with 
a  remarkable  facility  to  antispyhilitic  remedies.  Vomiting  is  quite 
frequent.  It  may  occur  without  food  in  the  stomach.  Vertigo  is  also 
a  common  symptom.  The  general  condition  of  the  patient  changes. 
He  becomes  apathetic,  somnolent,  languid,  loses  his  appetite  and  in  weight. 

In  cerebral  syphilis,  due  to  a  generalized  specific  arteritis,  the  symp- 
toms will  be  those  apoplexy  caused  by  arterial  lesions,  especially  by 
arterio-sclerosis.  Thrombosis  is  the  usual  occurrence.  In  such  cases 
hemiplegia,  aphasia  develop  slowly  after  a  prodromal  period  and  without 
loss  of  consciousness.  The  special  character  of  these  phenomena  is  that 
they  improve  rapidly  and  may  even  disappear  under  the  influence  of 
the  specific  treatment.  In  some  cases  there  are  brief  and  fugacious 
attacks  of  aphasia  without  paralysis  of  the  extremities.  Sometimes 
there  is  only  some  difficulty  of  articulating.  In  other  cases  the  attack 
consists  of  peculiar  sensations  (tingling,  numbness,  etc.)  on  one  side  or 
in  one  limb. 

Syphilitic  arteriti  may  affect  the  large  trunks  of  the  convexity  or 
at  the  base.  In  such  cases  the  entire  artery  may  become  obliterated  or  else 
aneurisms  may  form.  A  rupture  of  such  a  vessel  will  produce  a  sudden 
coma  and  death.  Generalized  or  focal  epileptiform  convulsions  are 
not  infrequent.  If  they  are  frequent  and  not  controlled,  psychic  mani- 
festations become  evident,  viz.  slow  cerebration,  loss  of  inhibition,  out- 
bursts of  anger. 

In  cerebral  syphilis  of  meningeal  origin  the  manifestations  are  dif- 
ferent from  those  of  the  previous  form.     When  the  meningitis  is  acute, 


340  SYPHILIS    OF    THE    NERVOUS    SYSTEM 

the  patient,  after  a  period  of  intense  headache,  becomes  stuporous. 
Hebetude  is  seen  in  his  acts  and  speech.  Coma  may  follow.  In  other 
cases  the  condition  may  be  reverse;  delirium,  excitement,  generalized 
convulsion  take  the  place  of  the  depression. 

In  basal  meningitis,  during  this  acute  stage,  very  frequently  palsies 
of  cranial  nerves  develop.  The  third  nerve  palsy  (ptosis,  strabismus) 
is  the  most  common  occurrence;  it  may  be  total  or  partial;  the  first  is 
rarer  than  the  latter.  When  the  optic  nerve  is  involved,  there  may  be 
amblyopia,  complete  blindness  or  hemianopsia.  Ophthalmoscopic 
examination  shows  the  following  possibilities:  optic  neuritis,  choked 
disc,  optic  atrophy.  The  fourth  and  sixth  nerves  are  sometimes  in- 
volved. Uhtoff  collected  only  twenty-seven  cases  out  of  150.  The  same 
can  be  said  of  the  fifth  nerve;  the  sensory  portion  is  more  frequently 
involved  than  the  motor.  The  seventh  and  eighth  are  occasionally  affected 
and  usually  together.  The  facial  palsy  is  usually  of  the  peripheral  type. 
It  may  be  associated  with  a  hemiplegia  or  the  opposite  side  (crossed 
paralysis).  It  occurs  when  the  facial  never  is  affected  with  other  struct- 
ures in  the  pons.  The  special  feature  of  these  palsies  lies  in  their  transitory 
character  and  in  the  fact  that  prompt  administration  of  antisphilitic 
treatment  is  followed  by  marked  improvement  or  even  complete  recovery. 

In  exceptional  cases  the  acute  stage  instead  of  improving  becomes 
prolonged:  the  patient  enters  rapidly  into  a  profound  coma  and  never 
regains  consciousness.  Among  the  less  frequent  symptoms  of  basal  menin- 
gitis may  be  mentioned:  polydipsia,  polyuria  and  glycosuria. 

In  acute  meningitis  of  the  convexity  epileptic  convulsions  will  figure 
prominently  instead  of  paralytic  symptoms.  They  may  be  generalized 
or  unilateral.  Charcot  believed  that  cortical  epilepsy  is  one  of  the  most 
frequent  consequences  of  syphilis  of  the  brain. 

In  chronic  syphilitic  meningitis,  in  addition  to  paralytic  or  epileptic 
phenomena,  there  will  be  psychic  disturbances.  They  consist  of  men- 
tal feebleness  associated  with  emotionality,  amnesia,  apathy  with  semi- 
somnolence,  automatism,  impairment  of  memory  and  in  advanced  cases 
of  dementia.     Delusions  and  hallucinations  may  also  be  present. 

In  cases  of  circumscribed  gummata  the  symptoms  depend  upon 
their  localization.  Whether  at  the  base,  on  the  convexity  or  in  the  sub- 
stance of  the  brain  the  symptomatology  will  be  that  of  tumors  of  the  brain 
(see  this  chapter). 

Syphilitic  Arteritis 

While  every  case  of  syphilitic  meningitis  is  associated  with  some 
degree  of  endarteritis,  there  are  nevertheless  cases  which  present  symp- 
toms of  arteritis  without  meningitis. 


SYPHILIS    OF    THE    NERVOUS    SYSTEM  341 

They  occur  most  frequently  according  to  Gowers  within  the  first  two 
years  after  the  infection  and  in  young  individuals. 

Symptoms. — Headache  exists  for  a  long  time  before  other  symptoms. 
It  is  usually  not  very  severe.  It  is  diffuse,  it  disappears  and  reappears; 
it  is  increased  by  mental  effort.  Short  attacks  of  dizziness  are  common. 
Sleep  is  usually  poor.  Psychic  symptoms  are  especially  conspicuous, 
viz.,  incapacity  for  work,  mental  fatigue,  feebleness  of  intelligence,  somno- 
lence, periods  of  excitement,  irritability. 

Besides,  a  typical  neurasthenic  symptom-group  is  not  infrequently 
observed  in  the  course  of  syphilis.  The  symptoms  are,  generally  speaking, 
those  of  neurasthenia  from  other  causes,  except  the  following  features: 
In  ordinary  neurasthenia  there  are  only  paresthesia?,  such  as  numbness, 
heaviness,  etc.  In  syphilis  there  is  pain,  which  is  worse  at  night.  Head- 
ache of  neurasthenia  is  usually  diurnal,  in  syphilis  nocturnal.  Antisyphilitic 
remedies  will  relieve  the  specific  headache,  but  not  the  headache  of  neuras- 
thenia. Finally  the  posterior  Wasserman  reaction  in  syphilis  and  the 
negative  one  in  neurasthenia  will  decide  the  diagnosis. 

Apoplectic  seizures  of  various  degrees  occur;  they  may  occur  even  dur- 
ing sleep.  The  seizure  is  usually  transitory.  Sudden  impediment  or  loss 
of  speech  may  occur  very  frequently.  Transitory  hemiplegia  or  mono- 
plegia or  else  a  sensory  disturbance  on  one  side  of  the  body  may  occur  and 
last  even  a  few  minutes. 

The  symptoms  of  endarteritis  just  enumerated  are  due  to  a  general 
thickening  of  the  intima,  producing  a  partial  occlusion  of  the  blood 
vessels.  These  changes  may  eventually  terminate  in  thrombosis  of  the 
diseased  vessels  and  produce  a  permanent  loss  of  function.  Most 
serious  symptoms  occur  when  a  basilar  artery  becomes  occluded;  a  fatal 
termination  is  almost  inevitable. 

Syphilitic  Hemiplegia 

Closely  associated  with  the  subject  of  syphilitic  arteritis  is  syphilitic 
hemiplegia.  The  latter  is  of  very  frequent  occurrence  and  therefore  of 
practical  importance. 

Etiology. — It  affects  particularly  individuals  who  have  not  been  treated 
or  imperfectly  treated,  also  in  cases  of  severe  infection.  It  occurs  at 
any  period  of  the  disease,  but  especially  between  three  and  fifteen  years 
after  the  infection.  It  may  coincide  with  the  secondary  mainfestations 
of  syphilis.  Men  are  more  frequently  affected  than  women.  x\s 
predisposing  causes  may  be  mentioned:  mental  and  physical  fatigue, 
violent   emotions   and   various   infectious   diseases  other  than  syphilis. 


34 2  SYPHILIS    OF    THE    NERVOUS    SYSTEM 

Hereditary  syphilis  plays  a  large  part  in  syphilitic  hemiplegia;  it  may 
occur  as  early  as  the  age  of  five  months  and  as  late  as  thirty-three  years. 
The  classical  infantile  spastic  hemiplegia  has  in  a  great  many  cases  a 
syphilitic  origin. 

Pathology.— The  chief  lesion  is  syphilitic  arteritis.  The  syphilitic 
virus  has  a  special  predilection  for  the  cerebral  arteries.  The  syphilitic 
process  either  narrows  or  obliterates  completely  the  lumen  of  the  blood 
vessel,  producing  either  a  temporary  cerebral  ischemia  or  softening,  or  else 
a  destruction  of  the  arterial  wall  followed  by  a  hemorrhage.  Among  all 
the  arteries  of  the  brain,  those  of  the  base  are  the  seat  of  predilection. 
The  terminal  branches  are  less  frequently  affected.  In  order  of  frequency 
the  following  arteries  are  involved:  Sylvian,  anterior  cerebral,  basilar 
trunk,  posterior  cerebral,  finally  the  branches  of  the  Sylvian  artery.  The 
main  syphilitic  lesion,  gumma,  may  form  in  the  arterial  walls  round  and 
hard  tuberosities,  but  more  frequently  the  syphilitic  process  consists  of 
plaques,  of  diffuse  infiltration.  The  artery  loses  its  transparency,  the 
plaques  form  a  thickened  prominence  within  and  without  the  artery. 
The  lumen  is  gradually  being  narrowed  so  that  it  presents  but  a  small 
fissure,  or  it  may  be  totally  obliterated  by  a  secondary  process,  viz.  throm- 
bosis. In  other  cases  the  artery  is  dilated  at  a  certain  distance  and  thus 
aneurisms  are  formed.  The  latter  are  observed  especially  in  the  basilar 
and  Sylvian  arteries.  Microscopically  the  following  condition  is  found: 
thickening  of  the  inner  wall,  leucocytic  infiltration;  the  elastic  inner 
membrane  is  fragmented,  torn;  the  adventitia  is  always  altered  and  the 
same  leucocytic  infiltration  is  observed.  The  muscular  layer  is  the  least 
involved. 

The  process  that  follows  is  the  terminal  state.  It  consists  of  chronic 
arteritis,  of  a  sclerotic  transformation:  the  entire  vascular  wall  became 
a  dense  fibrous  tissue.     Such  vessels  are  easily  ruptured. 

The  cerebral  tissue  in  view  of  the  damage  to  the  arteries  undergoes 
either  softening  or  suffers  from  hemorrhage.  The  first  is  the  most 
frequent. 

The  lesions  in  hereditary  syphilis  are  precisely  the  same  as  just 
described. 

As  to  the  spirochete,  there  are  very  few  cases  reported  in  which  they 
were  found  in  the  nervous  tissue. 

Symptoms. — Premonitory  symptoms  may  appear  weeks,  months  or 
years  before  the  hemiplegia. 

Headache  is  constant,  it  is  of  nocturnal  type  and  is  ameliorated  by 
specific  treatment.  At  the  same  time  the  individual  becomes  irritable 
and  depressed;  all  sorts  of  paresthesias  appear.     Two  special   symptoms 


SYPHILIS    OF    THE    NERVOUS    SYSTEM  343 

make  their  appearance,  viz.  deafness  or  vertigo,  also  ophthalmic  migraine. 
Following  an  effort  and  sometimes  without  cause  an  apoplectic  attack 
occurs.  It  may  be  only  slight  with  or  without  aphasic  symptoms,  or 
else  it  may  be  typical.  Irrespective  of  the  degree  of  involvement,  the 
attack  is  usually  transitory  and  lasts  but  a  few  days.  On  the  other 
hand  it  may  repeat  itself  in  the  same  day  or  every  few  weeks  or  months. 
When  later  on  the  hemiplegia  has  a  tendency  to  become  permanent,  it 
establishes  itself  by  gradation:  first  the  lower  limb,  a  few  days  later  the 
upper  limb  and  then  the  face. 

In  another  form  syphilitic  hemiplegia  is  associated  with  a  palsy  of 
cranial  nerves,  especially  the  third  nerve. 

Finally  syphilitic  hemiplegia  may  develop  suddenly  without  premoni- 
tory symptoms  similar  to  ordinary  hemiplegias.  Syphilitic  hemiplegia, 
when  definitely  established,  may  present  several  clinical  varieties  according 
to  the  intensity  of  the  motor  disturbances  and  to  the  localization  of  the 
lesion.  They  do  not  differ  from  hemiplegias  with  other  etiological  factors 
(see  chapter  on  Hemiplegia). 

Course,  Termination,  Prognosis.— In  cerebral  syphilis  with  a  general- 
ized specific  arteritis  the  attacks  of  hemiplegia  or  of  cranial  nerve  palsies 
may  be  fugacious  and  brief;  they  may  disappear  and  reappear  or  else  dis- 
appear completely  when  under  treatment.  On  the  other  hand  because  of 
repetition  of  attacks  the  damage  done  to  the  cerebral  tissue  or  to  the  cra- 
nial nerves,  especially  in  the  meningitic  forms  of  cerebral  syphilis,  may 
be  so  intense  that  the  lesion  remains  permanent.  Changes  in  the  cranial 
nerves  may  become  so  profound  (atrophy)  that  recovery  is  impossible. 
The  same  is  observed  in  circumscribed  gummata  on  the  surface  of  the 
brain.  Therapeutic  intervention  (salvarsan,  mercury,  iodides)  undoubt- 
edly modifies  considerably,  and  sometimes  favorably,  the  course  of  the 
disease,  but  it  is  powerless  in  cases  of  hemorrhages  and  softening  in  the 
cerebral  tissue.  The  prognosis,  generally  speaking,  is  good  when  the 
disease  is  treated  early,  but  it  is  grave  in  advanced  cases.  It  is  good  also 
when  the  Wasserman  reaction  becomes  negative.  It  is  grave  especially 
in  cases  with  epilepsy,  in  thrombosis  of  the  medulla  and  in  cases  with  pro- 
nounced mental  symptoms. 

Cerebral  syphilis,  as  a  rule,  is  a  serious  disease. 

Diagnosis. — The  chief  characteristics  upon  which  a  diagnosis  of  cere- 
bral syphilis  can  be  based  are  as  follows: 

1.  Sudden  onset  of  cerebral  symptoms  in  an  individual  in  the  midst  of 
apparently  good  health. 

2.  Headache  of  a  special  form  (nocturnal  exacerbation). 

3.  Palsies  of  cranial  nerves. 


344  SYPHILIS    OF    THE    NERVOUS    SYSTEM 

4.  Hemiplegia,  monoplegia,  focal  or  generalized  epilepsy. 

5.  The  course  of  the  disease:  disappearance  and  reappearance  of 
symptoms,  their  brief  duration;  multiplicity  of  symptoms. 

6.  Disappearance  or  prompt  amelioration  of  symptoms  under  the 
influence  of  salvarsan,  mercury  and  iodides. 

7.  The  exclusive  presence  of  lymphocytes  and  increase  of  albumen 
in  the  cerebro-spinal  fluid,  positive  Wasserman  reaction  in  the  cerebro- 
spinal fluid  and  in  the  blood  (see  chapter  on  Lumbar  Puncture). 

8.  History  of  syphilitic  infection. 

As  to  the  differential  diagnosis  with  paresis  and  tabes  see  the  respective 
chapters. 

SYPHILIS  OF  THE  SPINAL  CORD 

Spinal  syphilis  may  present  itself  in  the  following  forms:  (1)  chronic 
syphilitic  meningitis;  (2)  meningo-myelitis;  (3)  acute  syphilitic  myelitis; 
(4)  Erb's  spinal  paralysis;  (5)  gummatous  tumor;  (6)  syphilitic  disease  of 
the  vertebras,  which  is  very  rare. 

Pathology . — In  the  majority  of  cases  of  spinal  syphilis  the  lesion  is  a 
meningo-myelitis. 

As  the  point  of  departure  in  spinal  syphilis  is  usually  in  the  meninges, 
it  is  important  to  consider  first  the  changes  of  the  latter. 

The  most  frequent  lesion  of  the  membranes  is  pachymeningitis.  The 
thickened  dura  adheres  to  the  pia-arachnoid  and  through  the  latter  to 
the  cord.  It  may  be  diffuse  or  circumscribed.  The  cervical  region  is 
most  frequently  affected.  Histologically,  in  early  stages  the  typical 
specific  infiltration  will  be  found  (see  Cerebral  Syphilis),  but  in  an  ad- 
vanced stage  of  the  disease  there  is  a  fibrous  meningitis  consisting  of  a 
uniform  sclerotic  (connective)  tissue.     Solitary  gummata  are  rare. 

In  meningo-myelitis  (the  most  common  occurrence)  the  following 
lesions  are  usually  found. 

Acute. — The  pia-mater  is  infiltrated  with  round  cells  and  is  therefore 
thickened;  its  small  blood  vessels  are  the  point  of  orgin  for  this  infiltration. 
This  can  be  traced  in  all  the  prolongations  of  the  pia.  In  the  gray 
matter  of  the  cord  the  cell  infiltration  of  the  blood  vessels  is  at  its  maxi- 
mum. All  the  vessels,  arteries  and  veins  in  the  cord  are  similarly  affected. 
Syphilis  has  a  special  predilection  for  blood  vessels.  Thickening  of  the 
walls  of  the  latter  causes  a  narrowness  of  their  lumen,  hence  poverty  of 
blood  supply  and  softening  of  nervous  tissue.  Degeneration  and  atrophy 
of  cells  (especially  in  the  anterior  cornua)  follow.  In  the  white  matter  the 
nerve  fibers  at  first  lose  their  myelin  and  later  disappear;  they  soon 
become  substituted  by  proliferated  neuroglia. 


SYPHILIS    OF    THE    NERVOUS    SYSTEM 


345 


Chronic. — Cases  of  long  standing  are  characterized  by  sclerosis,  which 
affects  the  meninges  and  the  cord.  The  entire  section  of  the  cord  gives 
the  impression  of  a  fibrous  tissue.  Ascending  and  descending  degenera- 
tions are  seen  parting  from  the  original  focus. 

Symptoms. — In  the  majority  of  cases  the  meninges  are  first  affected 
and  the  cord  follows  (meningo -myelitis).  The  meningeal  symptoms 
precede  often  the  symptoms  of  myelitis  by  days  and  weeks. 


■.  ■ 


-  vOS: —  *&-■ 


V- 


y     : 


s 


Fig.  113. — Syphilitic  Meningo-myelitts.     (Flatau,  Jacobson,  Minor.) 

Pain  along  the  spine  radiating  toward  the  limbs  is  the  first  symptom. 
It  is  usually  aggravated  at  night.  It  is  due  to  involvement  of  the  meninges 
and  of  the  roots.  Numbness  and  tingling  (paraesthesiae)  in  the  limbs 
usually  accompany  the  pain.  Soon  the  cord  becomes  involved.  Then 
develops  paralysis  in  both  or  in  one  of  the  limbs,  particularly  in  the  lower, 
and  usuaUy  in  one  more  than  in  the  other.  It  is  flaccid  at  the  beginning, 
but  later  spasticity  develops.  The  sensations  are  usually  disturbed. 
Anaesthesia  or  hypassthesia  may  occur.  Sometimes  there  is  a  sensory  dis- 
sociation like  in  syringomyelia.  The  sphincters  are  always  and  very  early 
affected :  incontinence  is  rare.  The  sexual  power  is  impaired.  Muscular 
atrophy  may  occur,  but  this  is  exceptional. 

The  characteristic  feature  of  syphilitic  meningo-myelitis  lies  in  the 
multiplicity  of  symptoms,  in  their  unequal  distribution  on  both  sides  of 


346  SYPHILIS    OF    THE    NERVOUS    SYSTEM 

the  body,  in  their  variability  and  instability,  in  their  disappearance  and 
reappearance,  finally  in  their  modification  when  the  patient  is  under 
treatment.  A.  Thomas  considers  as  one  of  characteristic  signs  of  spinal 
syphilis  what  he  calls  "intermittent  spasm  of  the  blood  vessels  of  the 
spinal  cord."  The  latter  is  manifested  in  transitory,  mobile  disturbances 
in  sensations,  motion  and  function  of  the  bladder.  He  considers  these 
symptoms  as  the  premonitory  period  of  syphilitic  paraplegia.  (See  also 
spinal  intermittent  claudication  of  Dejerine  on  page  334.  According  to 
the  predominant  localization  of  the  lesion  spinal  syphilis  may  assume  the 
form  of  almost  any  of  the  cord  diseases:  transverse  myelitis,  tabes,  ataxic 
paraplegia,  etc. 

When  the  disease  advances  in  spite  of  the  treatment  (sign  of  well- 
established  and  irreparable  degenerative  lesions),  the  patient  is  in  the 
same  condition  as  in  chronic  myelitis  with  an  array  of  serious  symptoms, 
such  as  bed-sores,  incontinence,  contractures,  etc. 

Meningo-myelitis  of  the  thoracic  region  is  the  most  frequent  occur- 
rence. Cervical  meningo-myelitis  is  frequently  associated  with  a 
basal  meningitis.  Here  all  four  extremities  are  involved,  and  if  the 
eighth  cervical  and  first  dorsal  segments  are  affected,  symptoms  of  the 
sympathetic  will  be  present,  viz.  contraction  of  the  pupil,  enophthalmos, 
disturbance  of  secretion  of  the  sweat  glands.  Meningo-myelitis  of 
the  lurribo -sacral  region  will  present  a  flaccid  paralysis  with  loss  of  all 
reflexes,  severe  sensory  disturbances  in  the  lower  extremities,  disturb- 
ances in  the  function  of  the  bladder,  rectum  and  genital  organs.  Meningo- 
myelitis  may  also  present  the  Brown-Sequard's  type  of  paralysis  (see 
this  chapter  on  page  283).  Finally  the  roots  of  cauda  equina  may  be 
affected  with  gummatous  meningitis,  producing  a  complex  symptom- 
group  which  is  most  frequently  unilateral  (see  page  291). 

Erb's  Spastic  Spinal  Paralysis. — This  form  is  quite  common.  It  is 
characterized  pathologically  by  a  primary  degeneration  of  the  postero- 
lateral tract,  also  ascending  cerebellar  tract.  Shortly  after  the  initial 
chancre  (in  one  of  my  cases  six  months)  gradually,  but  progressively, 
paralysis  develops  in  the  lower  extremities.  There  are  no  marked  men- 
ingeal or  root  symptoms  observed  in  the  preceding  form.  Pain,  if  present, 
is  very  slight.  Girdle-sensation,  numbness  and  a  diminution  of  the 
general  sensibility  are  usually  present.  The  gait  appears  to  be  spastic, 
but  it  contrasts  strikingly  with  the  real  flaccidity  of  the  muscles  (hypo- 
tonia). There  is  no  muscular  atrophy.  The  knee-jerks  are  exaggerated, 
ankle-clonus,  Babinski's  sign,  Oppenheim's  and  paradoxical  reflexes  are 
elicited.  The  sphincters  are  early  and  constantly  involved.  The 
upper  extremities  are  usually  intact.     There  are  no  pupillary  changes. 


SYPHILIS    OF    THE    NERVOUS    SYSTEM  347 

Course,  Termination,  Prognosis. — Spinal  syphilis  is  rarely  acute. 
The  majority  of  cases  are  chronic.  They  may  last  years.  Complete 
recovery  is  not  frequent,  although  it  may  occur.  Those  cases  in  which 
evidences  of  well-established  tract  lesions  are  present  bear  a  bad  prognosis. 
It  is  also  unfavorable  in  cases  which  show  only  slight  improvement  in 
spite  of  a  prolonged  energetic  treatment.  Under  treatment  acute  symp- 
toms may  disappear,  such  as  pain,  bladder  disturbance,  but  disturbances 
of  reflexes,  of  gait,  may  persist.  Death  occurs  from  some  intercurrent 
disease  or  from  septic  infection  following  cystitis  or  bed-sores. 

Diagnosis. — The  unequal  distribution,  the  instability,  the  disap- 
pearance and  reappearance  of  the  symptoms,  their  prompt  amelioration 
when  under  treatment,  finally  their  disseminated  character,  are  sufficiently 
typical  in  the  majority  of  cases  to  make  a  diagnosis. 

Acute  Syphilitic  Myelitis. — Acute  myelitis  may  develop  as  a  direct 
consequence  of  syphilitic  infection. 

Pathology. — Swelling  of  lymph  sheaths  in  the  vessels  of  the  meninges 
and  cord,  rupture  of  small  vessels  and  extravasation  of  blood  corpuscles; 
degeneration  of  white  and  gray  matter. 

Symptoms. — Sudden  or  rapid  onset  with  the  following  premonitory 
signs:  paresthesia  and  sharp  pains  in  the  lower  limbs;  stiffness  of  the 
back;  cramps  in  the  legs;  retention  of  urine  and  feces;  the  reflexes  are 
variable.  Soon  appear  the  following  manifestations.  Complete  para- 
plegia. Profound  sensory  disturbances.  In  myelitis  of  the  lumbar  seg- 
ment the  tendon  reflexes  are  lost,  in  myelitis  of  the  dorsal  region  the 
reflexes  may  be  increased.  The  function  of  the  sphincters  is  very  early 
involved.     Wassermann  test  is  strongly  positive. 

Course. — Improvement  may  occur  rapidly  after  the  treatment  is 
instituted,  but  recurrences  are  frequent.  In  some  cases  it  may  terminate 
fatally  in  a  short  time. 

CEREBROSPINAL  SYPHILIS 

A  simultaneous  involvement  of  the  brain  and  the  spinal  cord  is  by 
far  more  frequent  in  syphilis  of  the  nervous  system  than  an  isolated  affec- 
tion of  each  of  the  two  portions  of  the  central  nervous  system.  In  the 
majority  of  cases  the  cerebral  disturbances  are  more  marked  than  the 
spinal;  the  reversed  condition  may  occasionally  occur. 

Not  infrequently  one  series  of  symptoms  disappears  promptly  when 
the  treatment  is  energetic,  or  else  some  symptoms  are  so  slight  that 
they  are  overlooked.  When  the  patient  comes  under  observation,  only 
the  spinal  or  only  the  cerebral  type  may  be  present.     A  close  investiga- 


348  SYPHILIS    OF    THE    NERVOUS    SYSTEM 

tion  will  reveal  in  a  large  number  of  cases  the  syphilitic  invasion  of  the 
entire  cerebro-spinal  axis  with  predominance  of  either  cerebral  (more 
frequent)  or  spinal  symptoms. 

Syphilis  of  the  Peripheral  Nerves. — In  spinal  syphilis  the  roots  are 
compressed  by  the  thickened  meninges  through  which  they  pass.  The 
nerve-trunks  in  their  course  between  the  roots  and  their  terminations 
may  encounter  syphilitic  gummata  in  the  tissues  and  undergo  compres- 
sion. But  the  peripheral  nerves  may  be  primarily  affected  by  the  specific 
poison,  resulting  in  s\p>hilitic  endo-  and  perineuritis  with  obliteration  of 
the  blood  vessels  and  subsequent  degeneration  of  the  nerve-fibers. 

Symptoms. — They  are:  neuralgia,  neuritis,  multiple  neuritis  and  root 
neuritis.  Syphilitic  neuralgia  occurs  in  the  early  stages  of  the  disease. 
Among  all  the  cranial  nerves  the  fifth  is  particularly  a  frequent  localization. 
Of  the  cervical  plexus  the  occipitales  are  often  affected.  The  intercostal 
nerves  are  not  rarely  involved.  Among  the  nerves  of  the  lumbo-sacral 
plexus  the  sciatic  suffers  most  frequently.  We  find  here  the  same  clinical 
manifestations  as  in  neuralgia  and  neuritis  of  any  origin;  the  same  motor 
and  sensory  symptoms  and  changes  of  reflexes.  Syphilis  rarely  affects  all 
nerves  with  equal  frequency.  Some  nerves  are  rarely  involved.  Thus, 
for  example,  the  seventh  nerve  palsy  is  not  a  frequent  occurrence  (see  my 
contribution  in  Arch,  of  Diagnosis,  1908). 

For  practical  purposes  it  is  important  to  bear  in  mind  that  if  in  any 
given  case  of  peripheral  nerve  involvement  of  long  standing,  after  having 
excluded  all  toxic  or  infectious  causes,  the  condition  persists,  syphilis  should 
be  thought  of  and  an  appropriate  treatment  instituted. 

Syphilis  and  Mental  Diseases. — Mental  disturbances  may  be  en- 
countered in  the  secondary  or  tertiary  periods.  Those  of  the  secondary 
period  are  genuine  psychoses  of  toxi-infectious  nature  in  this  sense,  that 
they  are  due  directly  to  the  action  of  the  specific  poison  and  not  to  cere- 
bral lesions  caused  by  syphilis.  They  usually  appear  at  a  time  when  the 
eruption  or  any  other  acute  symptoms  such  as  mucous  patches,  adenop- 
athies, etc.,  develop.  The  onset  is  usually  sudden.  Headache  and  in- 
somnia appear  first.  Hebetude,  stupor,  somnolence,  mental  obtusion, 
lack  of  orientation,  sometimes  delirium  and  hallucination  with  delusions 
of  persecution  appear  next.  These  are  the  phenomena  of  confusional 
insanity.  They  present  no  special  features  distinguishable  from  those  en- 
countered in  similar  states  caused  by  intoxications  or  infections  of  any  other 
origin,  except  with  regard  to  the  curative  effect  of  the  specific  treatment. 

The  psychoses  of  the  tertiary  period  differ  from  the  preceding  ones 
in  this  respect,  that  they  occur  long  after  the  initial  infection  and  do  not 
coincide  with  eruptions  or  other  secondary  manifestations.     They  are  due 


SYPHILIS    OF    THE    NERVOUS    SYSTEM  349 

to  meningoencephalitis,  obliterative  endarteritis  and  accompany  usually 
motor  symptoms,  such  as  epileptic  or  apopletic  attacks,  palsies  of  cranial 
nerves,  etc.  They  are  the  manifestations  of  cerebral  syphilis.  The 
mental  phenomena  of  the  latter  with  the  gradually  oncoming  dementia  as 
well  as  their  exacerbations  and  amelioration  when  under  treatment  have 
been  already  considered.     As  to  their  relation  to  Paresis  see  this  chapter. 

The  relation  of  hereditary  syphilis  to  mentality  is  of  great  impor- 
tance. With  the  advent  of  Wassermann  reaction  it  was  made  evident 
that  certain  organic  nervous  diseases,  especially  in  childhood,  are  the 
result  of  hereditary  syphilis.  It  is  admitted,  for  example,  that  juvenile 
paresis  is  due  to  hereditary  syphilis.  But  apart  from  organic  nervous 
diseases,  parental  syphilis  can  be  considered  also  as  the  cause  of  various 
mental  abnormalities,  such  as  imbecility,  idiocy,  of  various  psychoses  of 
the  young.  Such  individuals  not  infrequently  present  in  childhood  or 
at  puberty  some  external  manifestations  of  syphilis,  such  as  pupillary 
disorders,  iritis,  etc. 

Treatment.— Salvarsan,  mercury  and  iodides  are  the  chief  medications. 

Mercury, — There  are  three  methods  by  which  mercury  may  be  adminis- 
tered: (1)  inunction;  (2)  intra-muscular  injection;  (3)  ingestion. 

Inunction  is  a  very  good  method.  In  an  adult  5  j  of  unguentum  hy- 
drargyri  is  rubbed  in  twice  daily.  The  regions  of  the  body  having  most 
cellular  tissue  are  to  be  selected  for  the  inunctions.  The  upper  and  inner 
surface  of  the  arm  and  the  upper  and  inner  surface  of  the  thigh  are  pref- 
erable. Each  time  a  fresh  area  is  selected.  It  is  advisable  to  wash  off 
with  hot  water  and  soap  the  surface  to  be  rubbed  into.  Sulphur  baths 
are  advisable  during  the  mercurial  treatment,  as  thus  the  mercury  is  con- 
verted into  sulphate  of  mercury  which  is  easily  absorbed.  The  number  of 
rubbings  depends  on  the  intensity  of  the  symptoms  and  upon  the  tolerance 
of  the  individual.  I  am  in  a  habit  to  continue  the  inunctions  for  two 
weeks  in  succession,  if  there  are  no  symptoms  of  intolerance.  The  latter 
consist  of  salivation,  gastro-intestinal  disorders,  swelling  of  the  gums, 
febrile  state.     Each  inunction  should  last  twenty  or  thirty  minutes. 

Intra-muscular  injections  are  administered  in  soluble  or  insoluble 
forms  of  mercury.  The  latter  are  sufficient  once  a  week,  the  former  more 
frequently.  By  this  method  mercury  is  rapidly  absorbed.  It  is  indicated 
in  urgent  or  very  grave  cases. 

The  gray  oil  is  the  most  appropriate  preparation  among  the  insoluble 
ones;  biniodide,  benzoate  and  lactate  of  mercury,  among  the  soluble 
ones.  The  dorsal  muscles  or  the  gluteal  region  are  the  places  of  choice. 
The  injections  must  be  made  deeply  into  the  muscular  tissue  and  all  pos- 
sible antiseptic  precautions  must  be  taken  during  the  manipulation. 


350  SYPHILIS    OF    THE    NERVOUS    SYSTEM 

Finally  mercury  may  be  administered  by  the  mouth  when  inunc- 
tion or  intra-muscular  injections  cannot  be  used.  Among  all  prepara- 
tions I  prefer  bichloride  of  mercury  in  doses  of  gr.  1/32  t.  i.  d.  for  an 
adult. 

Mercurial  treatment  should  precede  the  iodides,  particularly  in  cases 
with  acute  symptoms.  The  mixed  treatment  is  appropriate  when  the  dis- 
ease becomes  fully  established  and  after  the  acute  symptoms  (headache, 
etc.)  have  been  partly  subdued  by  mercury.  For  the  dosage  of  iodides 
see  Treatment  of  Tabes.  The  action  of  the  drugs  is  more  prompt  in 
cerebal  than  in  spinal  symptoms.  In  cases  of  circumscribed  gummata, 
when  the  medical  treatment  has  failed,  operative  procedures  if  accessible 
should  be  resorted  to,  and  the  removal  of  the  tumor  be  followed  by  an- 
tisyphilitic  treatment. 

As  an  adjuvant  to  the  above  treatment  may  be  mentioned:  hydro- 
therapy, dietetic  and  hygienic  measures. 

Recently  Ehrlich  and  Hatta  have  discovered  a  new  arsenical  prepara- 
tion to  which  they  gave  the  name  of  606  and  later  of  salvarsan.  This 
has  proven  to  be  a  powerful  remedy  which  is  capable  to  destroy  the  spir- 
ochaetae  with  great  rapidity.  Its  usefulness  in  initial  chancre  and  secondary 
manifestations  is  beyond  question.  In  cerebro-spinal  syphilis  salvarsan 
has  also  been  of  considerable  value  but  not  to  the  same  extent  as  in  the 
preceding  conditions.  While  in  many  cases,  especially  of  cerebral  syphi- 
lis, it  succeeded  in  relieving  the  patient's  condition  with  respect  to  the 
frequency  and  intensity  of  the  short  transient  attacks,  nevertheless  in  a 
great  many  cases  it  did  not  prevent  recurrences. 

The  drug  may  be  introduced  into  the  body  by  deep  injections  in  the 
muscles  or  directly  into  the  circulation  by  intravenous  method.  The 
intravenous  method  presents  these  advantages,  that  there  is  no  pain  and 
the  elimination  is  rapid,  although  it  may  be  followed  sometimes  by  a  chill, 
rise  of  temperature,  nausea  and  vomiting. 

Dose  and  Method  of  Preparation. — Adult  average  dose  is  0.4  grm.  for 
women  and  0.6  grm.  for  men.  Dissolve  the  drug  in  100  c.c.  of  hot  sterile 
water,  add  drop  by  drop  a  15  per  cent,  solution  of  sodium  hydroxide 
(sterile  and  filtered)  and  agitate.  A  yellow  precipitate  will  form.  Con- 
tinue adding  the  solution  and  agitate  after  each  drop  until  solution  be- 
comes clear  again.  Then  add  sterile  hot  filtered  water  and  bring  total 
up  to  300  c.c.  Each  50  c.c.  corresponds  to  1  decigram  of  salvarsan. 
The  injection  is  to  be  given  at  ioo°  F. 

Very  recently  Ehrlich  has  recommended  a  modification  of  his  first 
discovery.  He  calls  the  new  preparation  neosalvarsan  or  914.  It  is 
given    in    doses    of   0.9   which   corresponds   to   0.6   of   salvarsan.     The 


SYPHILIS    OP    THE    NERVOUS    SYSTEM  351 

solution  is  made  by  simple  addition  of  the  substance  to  300  c.c. 
of  filtered,  distilled  water  at  room  temperature  and  injected  at  this 
temperature. 

In  treating  with  salvarsan  it  must  be  borne  in  mind  that  the  dose 
may  be  repeated  several  times.  This  should  be  done  when  the  symptoms 
are  only  slightly  influenced  or  especially  when  Wassermann  reaction  re- 
mains or  becomes  again  positive.  It  is  always  advisable  to  wait  after 
each  injection  several  weeks.  In  two  of  my  recent  cases  I  had  admin- 
istered three  injections  at  intervals  of  four,  six  and  eight  weeks  with  pro- 
gressive benefit. 

Contraindications. — Diseases  of  the  heart,  kidneys,  affections  of  the 
optic  nerves,  cachexia,  alcoholism,  advanced  degenerative  changes,  are 
the  main  contraindications. 

Unfavorable  By-effects. — The  following  complications  have  been  ob- 
served from  the  treatment  with  salvarsan. 

Cranial  nerve  involvement.  It  seems  that  there  is  a  special  predi- 
lection of  arsenic  for  the  eighth  nerve.  Not  only  salvarsan  but  also  other 
arsenical  preparations  show  the  same  characteristic  feature.  The  toxic 
effect  particularly  occurs  after  repeated  injections.  In  another  series  of 
cases  there  may  be  a  simultaneous  paralysis  of  several  cranial  nerves, 
such  as  seventh,  eighth  and  ocular  nerves.  In  one  of  my  cases  a  facial 
palsy  of  peripheral  type  occurred  six  weeks  following  a  second  injection 
of  salvarsan.  In  another  case  I  observed  two  days  after  the  first  injec- 
tion a  very  marked  myosis  of  the  left  pupil  which  remained  unaltered  two 
years  later. 

Heuser  {Mediz.  Klinik,  191 1)  reports  a  case  of  iritis  in  a  few  days 
after  intra-muscular  injection  of  0.2  grm.  followed  by  an  intra- venous  in- 
jection of  0.4  grm.  of  salvarsan,  also  a  case  of  paralysis  of  vocal  cords  and 
soft  palate  after  an  injection  of  0.5  grm.,  finally  a  case  of  choked  disc  with 
facial  paralysis.  In  the  first  two  cases  there  were  also  epileptiform  con- 
vulsions. Heuser  bases  his  belief  in  the  neurotropic  action  of  salvarsan 
also  on  the  fact  that  arsenic  could  be  detected  in  the  urine  (twenty-three 
cases)  nine  months  and  in  the  blood  two  months  after  the  injection. 
Finger  {fieri,  klin.  Wchn.,  191 1)  reports  three  cases  of  optic  neuritis  after 
salvarsan. 

In  a  third  group  of  cases  recurrences  of  nerve  disturbances  have  been 
observed  after  salvarsan  injections.  It  is  difficult  to  say  whether  the 
drug  is  directly  or  indirectly  the  cause  of  the  recurrences.  These  special 
neuro-reactions  are  still  debatable.  They  usually  occur  some  time  after 
the  injections.  It  is  possible  that  arsenic  has  a  special  irritative  local 
effect  on  the  spirochaetas. 


352  SYPHILIS    OF    THE    NERVOUS    SYSTEM 

Among  other  by-effects  may  be  mentioned:  cardiac  arrhythmia;  dis- 
turbed function  of  the  bladder,  especially  retention;  constipation;  diarrhoea; 
jaundice  (in  one  of  my  cases  it  appeared  on  the  day  following  the  first  in- 
jection); disturbances  of  tendon  reflexes;  peroneus  paralysis;  epileptifor 
seizures.  Fatalities  have  also  been  recorded.  They  may  occur  a  f  - 
days  or  severa1    •  JQks  after  the  administration  of  the  drug. 


CHAPTER   XXII 

PARESIS 

GENERAL  PARALYSIS  OF  THE  INSANE  (DEMENTIA  PARALYTICA) 

It  is  a  parasyphilitic  disease  characterized  pathologically  by  a  diffuse 
meningo-encephalitis  and  clinically  by  symptoms  of  progressive  dementia.1 


^ 


&f.- 


<x^ 


v:--  .- 


Fig.  114. — Old  Case  or  Paresis.     {Flatau,  Jacobson,  Minor.) 
a,  dura;  b,  pachymeningitis;  c,  capillaries;  d,  beginning  small  hematomata  in  the  dura; 
e,  leucocytic  infiltration  in  walls  of  blood  vessels  and  in  tissue  of  dura;  /,  new  membrane  with 
pigment. 

Pathology. — The  meninges,  brain,  skull  and  spinal  cord  are 
involved. 

Meningitis. — The  pia-arachnoid  is  inflamed,  thickened  and  adherent 

1  Our  former  view  concerning  the  relationship  of  Syphilis  to  Paresis  and  Tabes  must 
now  be  modified.  Noguchi  and  Moore  (/.  of  Exper.  Med.,  Feb.  1,  1513)  have  found  the 
spirochaetse  in  the  brains  of  12  paretics,  and  Noguchi  found  them  in  the  posterior  columns 
of  a  tabetic.  In  the  brain,  they  are  seen  more  numerous  in  the  cortical  than  in  the 
white  matter,  they  are  scattered  in  groups  between  the  cells  and  neuroglia  fibers.  They 
are  rare  in  the  vicinity  of  the  blood  vessels,  and  not  found  in  the  walls  of  the  latter.  These 
findings  have  been  corroborated  by  Levaditi,  Marie  and  Bankowski  {Presse  medic.  No.  36, 
19 13).     The  parasyphilitic  diseases  are,  therefore,  very  probably  true  syphilitic  affections. 

23  353 


354  PARESIS 

to  the  cortex.  When  attempt  is  made  to  detach  it  from  the  subjacent  tissue, 
portions  of  the  latter  are  torn  off.  The  most  favorite  seat  of  the  adhesions 
is  the  Rolandic  area  and  the  bases  of  the  frontal  convolutions.  The  walls 
of  the  blood  vessels  are  infiltrated  with  round  cells.  Serous  fluid  in  the 
ventricles  and  sub-arachnoid  space  is  increased  in  amount.  The  space  over 
the  optic  tracts  and  crura  has  a  milky  appearance. 

Encephalitis. — Macroscopically  the  convolutions  are  thin  and  shrunken, 
the  gray  substances  appear  softened  and  the  weight  of  the  entire  brain  is 
diminished.  Atrophy  is  particularly  observed  in  the  central  convolutions, 
basal  ganglia,  medulla  and  pons.  Softening  which  is  due  to  obliterations 
of  small  arterioles  is  diffuse  and  irregularly  distributed.  The  ventricles 
are  dilated  and  their  ependyma  is  congested. 


^v/v^§| 


>'*}■ 


Fig.  115.  — Paresis.    Transverse  Section  of  a  Cortical  Blood  Vessel,  Showing  Leu- 
cocytic  Infiltration.     (Bouchard  and  Brissaud.) 

Histologically  the  cells,  fibers,  neuroglia  and  blood  vessels  are  found 
altered. 

The  cortical  cells  are  in  a  state  of  degeneration.  In  acute  stage 
they  are  swollen,  the  chromatophilic  substance  disappears,  the  axis- 
cylinders  and  protoplasmic  processes  are  enlarged.  In  chronic  stage  the 
cells  are  sclerosed,  deformed  and  disintegrated.  The  neuro-fibrils, 
according  to  some,  are  pigmented  and  diminished  in  number. 

The  nerve-fibers  undergo  a  gradual  destruction.  At  first  they 
become  varicose  and  then  lose  their  myelin,  also  the  axis-cylinder.  The 
cortical  and  sub-cortical  fibers  are  particularly  affected.  The  neuro-fibrils 
of  the  cortical  cells  are  generally  affected.  The  intra-cellular  fibrils  are 
more  involved  than  the  extra-cellular,  also  those  of  the  small  pyramidal 
cells  more  than  those  of  the  large  pyramidal  ones. 

The  neuroglia  is  in  a  state  of  hyperplasia. 


PASESIS 


355 


The  changes  of  the  blood  vessels  consist  of  leukocytic  infiltration  of 
their  adventitia  and  thickening  of  the  other  coats,  so  that  eventually  they 
become  stenosed  and  completely  obliterated.  Similar  alterations  are 
found  in  the  lymphatic  perivascular  spaces. 

The  above  histological  alterations,  viz.  atrophy  of  neurons  and 
hyperplasia  of  vessels  and  connective  tissue,  are  found  not  only  in  the 
cortex,  but  also  in  basal  ganglia,  pons,  medulla  and  cerebellum,  although 
to   a   lesser    degree.      Some   of   the   cranial   nerves    show   atrophy   and 


Fig.   116.  — Paresis.    Longitudinal  Section  of  a  Cortical  Blood  Vessel,  Showing 
Leucocytic  Infiltration.     {Bouchard  and  Brissaud.) 

degeneration.  Optic  neuritis  and  atrophy  are  quite  frequent.  The 
third  and  sixth  are  not  rarely  involved.  Among  other  constant  patholog- 
ical lesions  should  be  mentioned  a  sclerosis  of  the  posterior  and  lateral 
columns  of  the  spinal  cord,  the  first  more  than  the  second  (Anglade, 
Wyruboff,  Klippel). 

Less  Constant  Lesions. — Pachymeningitis  (hemorrhagica) ;  thickening 
of  the  skull  with  obliteration  of  the  diploe  and  formation  of  exostoses; 
hyperemia  and  thickening  of  spinal  meninges  with  adhesions  to  the  verte- 
bras; proliferation  of  the  neuroglia  in  the  medulla  and  cord;  atrophy  of  the 
cells  of  the  anterior  cornua  in  the  cord  and  of  the  nuclei  of  the  cranial 


356  PARESIS 

nerves  (third,  sixth,  fifth,  seventh,  also  olfactory  nerve) ;  inflammation  of 
spinal  nerves.  The  sympathetic  nervous  system  has  also  been  found 
involved  in  paresis. 

The  peripheral  nerves  show  parenchymatous  degeneration,  atrophy  and 
proliferation  of  connective  tissue.  These  changes  are  more  frequently 
seen  in  the  lower  than  in  the  upper  limbs. 

The  cerebro-spinal  fluid  presents  the  following  characteristics: 
increase  in  the  amount,  lymphocytosis,  increase  of  albumen  (Nonne's 
Phase  I),  also  positive  Wassermann  reaction  (see  also  page  374). 

Symptoms. — Three  periods  can  be  considered,  viz.  (1)  initial  period, 
(2)  period  of  full  development,  (3)  terminal  period. 

1.  In  the  majority  of  cases  the  onset  is  very  slow  and  imperceptible. 
Gradual  changes  take  place  in  the  physical  and  intellectual  spheres 
before  the  typical  symptoms  begin  to  appear.  The  general  appearance 
of  the  patient  is  altered:  he  is  pale,  the  features  are  drawn  and  without 
expression.  Epileptic  seizures,  generalized  or  unilateral,  may  occur  long 
before  the  initial  period;  they  may  be  motor  or  sensory;  they  may  be  of 
petit  mal  or  grand  mal  character.  Transient  attacks  of  aphasia  are  not 
infrequent.  Palsies  of  ocular  muscles  (strabismus,  ptosis,  inequality  and 
irregularity  of  the  pupils,  diplopia)  are  common.  Sudden  loss  of  power 
in  one  limb,  increased  reflexes,  various  neuralgias,  gastric  and  vesical 
crises,  insomnia  are  also  not  infrequent.  Various  trophic  or  vaso -motor 
disturbances,  as,  for  example,  spontaneous  fractures,  falling  out  of  the 
hair  and  nails,  various  visceral  disturbances  (vomiting,  diarrhoea,  palpita- 
tion, etc.)  are  observed. 

Mentally  the  patient  is  changed.  He  is  irritable,  depressed,  indif- 
ferent. His  memory  is  weakened.  His  aptitude  for  work  is  diminished 
and  when  he  makes  an  attempt,  an  unusual  effort  is  required.  It  is 
difficult  to  hold  his  attention  in  a  conversation  or  transaction.  Briefly 
speaking,  he  presents  symptoms  usually  found  in  neurasthenia. 

Gradually  these  symptoms  increase  in  intensity  and  others  are  added. 
Instead  of  being  morose  and  apathetic  the  patient  may  be  restless  and 
excitable.  The  least  contradiction  angers  him.  He  becomes  egotistical 
and  his  moral  sense  is  blunted:  he  has  no  obligations  to  his  family,  lies, 
deceives.  Conventional  laws  are  beginning  to  be  ignored  by  him. 
Crimes  against  morality  are  of  common  occurrence. 

Sometimes  the  patient  shows  in  the  initial  stage  an  exalted  intel- 
lectual activity.  Instead  of  depression  he  presents  a  sense  of  well-being. 
There  is  a  hyperamnesia,  a  remarkable  power  of  forming  ideas,  of  creat- 
ing images,  which  is  noticeable  in  acts  and  speech.  He  plans  extrava- 
gantly, speaks  extravagantly  of  his  fortunes,  of  his  properties.     The  same 


paresis  357 

phenomenon  is  observed  in  various  vegetative  functions;  exaggerated 
appetite,  sexual  and  alcoholic  excesses  are  frequent. 

These  changes  in  the  intellectual  and  moral  spheres  are  forerunners 
of  oncoming  dementia.  Physical  signs  begin  to  become  conspicuous  at  this 
stage  of  the  disease.  An  awkwardness  in  gait,  in  station,  in  doing  fine 
work,  in  handling  objects,  a  slight  tremor  of  the  hands,  hesitation  of 
speech,  are  beginning  to  be  noticeable. 

2.  In  the  period  of  full  development  of  the  disease  the  physical  and 
psychic  symptoms  are  very  marked. 

Physical. — i.  Tremor  is  constant.  It  is  generalized,  but  more  marked 
in  the  hands.  It .  is  fine,  rapid  and  intentional.  Instead  of  tremor, 
there  may  be  only  jerky  movements  of  the  fingers,  which  are  noticeable 
upon  a  voluntary  act.  Tremor  and  instability  are  also  present  in  the 
tongue,  lips  and  muscles  of  the  face.  They  are  particularly  noticeable 
in  attempts  to  speak.  The  face  when  at  rest  gives  the  impression  of 
immobile  mask,  but  on  the  least  emotion  the  facial  muscles  commence 
to  contract  excessively  as  if  they  awaken  from  a  latent  spasm.  This 
mixture  of  paresis  and  spasm  is  quite  characteristic  of  general  paralysis 
of  the  insane.     It  demonstrates  a  disturbance  of  mimicry. 

2.  The  speech  is  characteristic.  It  may  be  tremulous  (ataxic)  or 
spasmodic.  In  the  first  case  the  words  are  precipitated,  then  interrupted 
and  then  again  continued,  at  the  same  time  hesitated  and  repeated. 
In  the  second  case  the  words  and  syllables  are  slowly  pronounced  and 
resemble  those  in  multiple  sclerosis.  There  is  a  dysarthria:  the  labials 
and  gutturals  are  particularly  affected. 

3.  The  disturbance  in  writing  is  analogous  to  that  of  the  speech: 
omission  of  letters,  syllables,  words,  mistakes  of  grammer,  repetition, 
poor  spelling  of  letters,  etc.,  are  all  typical.  In  an  advanced  period  the 
writing  is  not  distinguishable. 

4.  Visual  disorders  most  frequently  consist  of  myosis  or  mydriasis, 
irregularity  and  inequality  of  the  pupils  and  reflex  disturbance.  Argyll- 
Robertson  sign  is  frequent.  A  paradoxical  pupil  (response  to  light  and 
not  to  accommodation),  Piltz's  sign  (contraction  of  the  pupil  at  the 
attempt  to  close  eyelids)  may  occur.  Ocular  palsies,  diplopia,  nystagmus 
and  changes  in  the  eye  grounds  (optic  atrophy)  are  not  rare.  According 
to  Uhtoff  optic  atrophy  is  observed  in  50  per  cent,  of  paretics,  while 
Galezowski's  statistics  show  only  18  per  cent. 

5.  Muscular  weakness  is  contant.  The  face  is  mask-like,  without 
expression.  Sometimes  there  is  loss  of  control  over  the  facial  muscles 
and  the  masticated  food  or  liquids  run  out  of  the  mouth.  Paralysis, 
incomplete  or  transient,  after  an  apoplectic  seizure  is  frequent.     Attacks 


358  PARESIS 

of  monoplegia  or  hemiplegia,  of  aphasia,  of  blindness  or  deafness,  may 
occur.  In  the  majority  of  cases  they  are  temporary.  An  attack  of  this 
nature  may  be  accompanied  by  loss  of  consciousness.  Occasionally 
and  in  severe  cases  an  attack  may  leave  the  patient  permanently  hemi- 
plegic.  Spasmodic  contractions  are  met  with.  Epileptiform  seizures, 
while  more  frequent  in  the  first  period,  may  also  occur  at  this  stage. 
They  may  be  generalized  or  focal.  They  may  affect  the  sensory  motor 
and  vasomotor  centers  equally.  Instead  of  convulsive  attacks  there 
may  be  fits  of  petit  mal.  Each  attack  of  epileptiform  convulsions  leaves 
the  paretic  more  exhausted  than  in  ordinary  cases  of  epilepsy.  The 
gait  is  usually  ataxic ;  it  may  be  also  spastic.  Romberg's  sign  may  also 
be  present. 

6.  The  tendon  reflexes  are  altered  in  90  per  cent,  of  cases.  In  the 
majority  they  are  exaggerated,  in  a  small  percentage  of  cases  lost.  Bab- 
inski  sign  is  rare,  while  the  paradoxical  sign  is  frequent.  This  peculiarity 
lies  in  the  fact  that  the  pyramidal  tract  is  only  slightly  involved.  Bab- 
inski  sign  is  usually  the  expression  of  a  well-defined  degenerative  lesion, 
while  the  reflex  described  by  me  is  present  in  the  earliest  changes  of  the 
motor  pathway  (/.  of  Nervous  and  Ment.  Dis.,  1907).  Ankle-clonus 
and  Oppenheim's  reflex  are  rare. 

7.  Cutaneous  sensibility  is  usually  diminished.  Neuralgia,  migraine, 
paresthesias  may  be  met  with.  The  special  senses  may  be  affected. 
Anosmia,  changes  of  taste,  of  hearing,  are  not  rare. 

8.  Trophic  disturbances,  such  as  falling  out  of  the  nails,  of  the  teeth, 
herpes  zoster,  bed-sores,  arthropathies,  spontaneous  fractures,  are  some- 
times observed. 

Vasomotor  symptoms,  such  as  tinnitus  aurium,  pallor,  coldness, 
gangrene  of  the  extremities,  are  common.  There  is  a  tendency  to  for- 
mation of  hematomata.     Hematoma  of  the  ear  is  not  rare. 

9.  Visceral  Disturbances. — They  are:  increased  digestive  function 
in  the  exalted  state,  diminished  in  the  depressive  state,  gastro-enteritis ; 
pulmonary  involvement;  circulatory  disorders  (aortitis);  genito-urinary 
disturbances  (impotence,  sterility,  suppression  of  menses);  involvement 
of  the  sphincters  of  the  bladder  and  rectum  (retention  or  incontinence, 
frequent  or  imperative  micturition). 

10.  The  secretions  may  undergo  changes.  Excessive  salivation  and 
sweating  have  been  observed. 

1 1 .  The  toxicity  of  blood  and  it  bactericidal  power  are  increased. 

The  cerebro-spinal  fluid  contains  quite  a  perceptible  amount  of  fluid 
and  abundant  lymphocytes.  Wasserman  reaction  is  positive  and  there  is 
increase  of  albumen  (Nonne's  Phase  I). 


paresis  359 

12.  The  general  nutrition  as  a  rule  is  lowered  at  first,  then  gradually 
improves  and  the  patient  becomes  stout.  Later  on  there  is  again  a  loss 
which  finally  ends  in  cachexia. 

Psychic  Symptoms. — Progressive  enfeeblement  of  the  mental  faculties, 
viz.  progressive  dementia,  which  is  accompanied  by  symptoms  of  other 
psychoses,  is  characteristic  of  paresis. 

Amnesia  predominates.  In  the  initial  stage  the  loss  of  memory  af- 
fects recent  events.  In  the  second  period  it  concerns  also  old  events. 
The  patients  forget  even  their  age,  birthplace,  etc. 

Judgment,  power  of  attention,  will  power  are  all  markedly  impaired. 
The  patient  is  easily  influenced,  because  of  defect  in  the  will  power.  As 
mental  self-control  is  defective,  the  patient  succumbs  to  the  influence  of 
impulse.  Outbreaks  of  emotional  nature  are  then  seen.  Theft,  assaults, 
homicide,  etc.,  are  not  rare.     Performance  of  delicate  acts  is  deficient. 

A  very  important  symptom  is  the  inability  to  recognize  one's  own 
infirmity  in  spite  of  the  exuberant  spirit.  Such  a  patient  does  not  appre- 
ciate the  fact  that  he  is  ill,  that  he  fails  in  performing  his  work.  The 
want  of  knowledge  of  this  infirmity  leads  to  formation  of  ideas  of  self- 
importance,  of  expansive  ideas.  The  latter  are  very  common  in  paresis. 
The  expansion  or  exaltation  is  noticeable  in  psychic,  motor,  sensory  and 
vegetative  spheres.  The  patient  believes  himself  as  possessing  millions 
of  dollars,  of  properties,  of  friends,  of  enemies.  He  is  famous,  his  name 
is  mentioned  everywhere;  he  is  a  prophet,  a  god,  etc.  He  is  restless, 
does  not  sleep,  eats  abundantly,  shows  a  tendency  to  excesses  of  all  kinds. 
Gradually  the  moral  sense  becomes  obtunded.  The  patient  is  unable  to 
form  new  connected  ideas,  unable  to  orient  himself;  he  is  indifferent  and 
becomes  automatic  in  his  acts.  The  latter  are  foolish,  childish.  The 
further  the  dementia  advances,  the  less  he  is  capable  to  take  care  of  him- 
self; he  is  unclean  about  his  person,  indecent,  uses  profane  language 
irrespective  of  the  surroundings. 

During  the  progressive  development  of  the  dementia  other  symp- 
toms are  observed,  viz.  delirium,  confusion,  delusions  with  or  without 
hallucinations. 

The  delusions  in  paresis  are  usually  vague,  unsystematized,  unstable. 
Sometimes  they  are  systematized  and  fixed,  similar  to  those  of  paranoia. 
They  may  be  depressive,  persecutory  or  else  expansive.  They  may  be 
also  hypochondriacal.  Hallucinations  not  infrequently  accompany  the 
delusions.  Hallucinations  of  various  senses  are  not  rare  in  paresis,espe- 
cially  of  sight  and  hearing.  One  of  my  patients  had  an  olfactory  halluci- 
nation: he  smelled  feces  at  the  approach  of  food. 

A  delirious  state  with  great  excitement  and  restlessness  occurs  episodic- 


360 


PARESIS 


ally  during  the  entire  course  of  paresis.     A  confusional  state  is  present,  par- 
ticularly in  the  second  stage  of  the  disease. 

The  various  delusions  just  mentioned  may  sometimes  simulate  other 
psychoses,  but  they  never  present  the  characteristic  and  well-defined 
features  of  the  latter. 

3.  Terminal  Period. — The  physical  and  mental  symptoms  just  de- 
scribed progress  gradually  toward  a  terminal  stage,  which  is  character- 
ized by  complete  deterioration  and 
relaxation  of  sphincters.  The 
hebetude,  dementia,  difficulty  of 
speech,  pupillary  symptoms,  ataxia 
of  locomotion,  tremors,  have  all 
reached  the  climax.  New  phe- 
nomena develop.  Paralysis  of  the 
vesical  and  rectal  sphincters,  par- 
alysis of  the  pharynx,  trophic 
disturbances,  viz.  bed-sores,  render 
the  paretic  completely  helpless  and 
hasten  his  death.  Apoplectiform 
and  epileptiform  attacks  are  fre- 
quent at  this  stage  of  the  malady 
and  may  be  the  immediate  cause 
of  death.  Not  infrequently  death 
occurs  from  an  intercurrent  disease 
(pneumonia  or  others). 

Forms. — (a)  Simple  De- 
mented Form. — It  is  very  fre- 
quent. It  is  characterized  besides 
the  typical  physical  symptoms  by 
a  gradually  developing  dementia 
from  the  beginning  to  the  end  and 
not  accompanied  by  delusions.  The  dementia  is  usually  not  marked, 
so  that  the  patient  continues  for  a  certain  period  his  usual  occupation. 
Epileptic  seizures  are  very  frequent  in  this  form  of  paresis. 

(b)  Depressive  Form./ — Marked  mental  depression  is  characteristic. 
Delusions  of  persecutory  or  hypochondriacal  nature  are  present.  In  some 
cases  the  latter  are  so  pronounced  that  melancholia  may  be  thought  of, 
especially  when  the  ideas  of  unpardonable  sin  or  impending  evil  and 
tendency  to  suicide  are  predominating.  However  these  delusive  ideas 
are  unstable  and  do  not  present  on  a  whole  the  picture  of  genuine  melan- 
cholia.    In  this  form  there  is  a  marked  tendency  to  obesity. 


Fig.  117. — Depressive  Form  of  Paresis. 
Expression  Sad,  Preoccupied  and  Demented 
(Bouchard  and  Brissaud.) 


PARESIS 


361 


Remissions  are  not  so  frequent  as  in  the  following  form. 

(c)  Expansive  Form. — It  is  characterized  by  an  exalted  general  atti- 
tude. Even  in  the  absence  of  distinct  delusions  the  patient's  manner 
and  expression  betray  a  self-satisfaction.  The  paretic  is  happy,  contented 
with  everybody  and  everything.  Sometimes  the  expansion  may  increase 
to  such  an  extent  as  to  cause  marked  restlessness  and  excitement,  so  as  to 
simulate  mania.  However,  taken 
as  a  whole,  it  is  not  a  genuine 
mania.  As  to  the  delusions,  they 
are  of  the  exalted  type.  The  pa- 
tient is  a  multimillionaire,  prophet, 
god,  great  actor,  writer,  etc.  (see 
above).  In  spite  of  his  great  self- 
importance,  he  is  very  easily  in- 
fluenced and  is  submissive. 

(d)  Circular  Form. — It  is  char- 
acterized by  alternation  of  depres- 
sive and  expansive  states.  It 
"may  be  confounded  with  the 
manic-depressive  psychoses.  An 
examination  for  physical  signs  and 
repeated  observation  will  decide 
the  diagnosis. 

(e)  Galloping  Form. — In  rare 
cases  rapid  evolution  of  symptoms 
is  observed.  The  mental  mani- 
festations   are    characterized    by 

'rapid  deterioration  of  mind,  loss 
of  control  of  the  sphincters,  bed- 
sores, exhaustion  and  death.  It 
is  possible  that  the  symptoms  of 
paresis   in    such    cases    had  been 

long  at  work  and  because  of  mildness  of  the  manifestations  they  were 
overlooked.  Under  the  influence  of  some  exciting  cause,  the  symptoms 
suddenly  assumed  a  rapid  course  and  promptly  reached  a  fatal  termination. 
(/)  juvenile  Paresis. — It  is  occasionally  observed.  In  one  of  my 
cases,  a  young  man  of  eighteen,  the  symptoms  began  to  show  themselves 
at  the  age  of  twelve.  Signs  of  mental  enfeeblement  appeared  first. 
Speech  disturbances  of  the  typical  paretic  variety  developed  gradually 
two  years  later.  At  the  time  I  saw  him,  he  presented  the  characteristic 
fine  tremor,  paretic  speech,  ataxia,  exaggerated  knee-jerks,  irregular  and 


Fig.  118. — Expansive  Form  of  Paresis.     Ex- 
pression Happy.     (Bouchard  and  Brissaud.) 


362  PARESIS 

unequal  pupils  and  depression  with  hypochondriacal  delusions  without 
hallucination.  He  never  had  epileptic  or  apoplectic  seizures.  He  is  still 
living,  but  his  dementia  is  getting  progressively  deeper.  Wassermann 
reaction  was  strongly  positive. 

Course,  Duration,  Prognosis. — Paresis  is  essentially  a  progressive 
disease.  Its  course  may  be  slow  or  rapid.  The  course  of  the  simple 
demented  form  is  extremely  slow.  The  expansive  form  runs  a  more 
rapid  course  than  the  depressive  form.  Complications,  such  as  apoplec- 
tiform or  epileptiform  seizures,  visceral  complications,  hasten  the  course 
of  the  disease. 

Paresis  may  present  periods  of  arrest  and  improvement,  so-called 
remissions.  The  latter  consist  of  a  partial  or  complete  disappearance 
of  the  mental  manifestations.  These,  so  to  speak,  lucid  intervals  may  oc- 
cur at  any  of  the  three  periods  of  the  disease.  They  may  last  from  a  few 
days  to  weeks,  months  and  even  years.  They  are  usually  the  result  of 
a  proper  and  early  instituted  treatment.  Remissions  sometimes  follow 
a  traumatism  or  a  protracted  suppuration.  In  the  majority  of  cases 
the  physical  symptoms  persist.  I  have  seen  cases  in  which  very  great 
improvement  of  the  physical  signs  also  occurred  during  remissions.  The 
duration  of  paresis  is  from  a  few  months  to  several  years  (five  and  ten 
years).  The  second  stage  is  the  longest.  The  prognosis  is  unfavorable. 
Death  may  result  from  the  invariably  progressive  course  of  the  disease, 
ending  either  with  a  disease  of  the  lungs  (tuberculosis,  pneumonia),  diar- 
rhoea, bed-sores  or  extreme  exhaustion.  It  may  also  end  accidentally: 
suffocation  from  impaction  of  food  in  the  larynx  during  an  epileptic 
seizure;  fractures  of  bones;  finally  suicide. 

Sudden  death  has  also  been  observed.  In  such  cases  either  there  is 
an  element  of  alcoholism,  or  a  cardiac  pathological  condition,  or  a  cerebral 
hemorrhage.  In  some  rare  cases  no  perceptible  cause  could  be  found. 
Sudden  death  occurs  more  frequently  in  middle  aged  individuals  than  in 
elderly  or  young  persons. 

Diagnosis. — As  the  onset  and  various  forms  of  paresis  may  simulate 
other  diseases,   the  differential  diagnostic  points  must  be  emphasized. 

The  neurasthenoid  state  of  the  first  period  will  be  distinguished 
from  genuine  neurasthenia  by  the  following  considerations: 

A  neurasthenic  is  capable  of  presenting  a  detailed  account  of  his  ills. 
He  exaggerates  his  symptoms,  it  is  true;  nevertheless  they  are  recited 
in  the  most  connected  manner.  The  patient  repeats  his  recital  often, 
but  this  repetition  is  not  because  of  a  genuine  amnesia,  but  because  he 
is  anxious  to  analyze  his  troubles  and  to  draw  his  physician's  special 
attention  to  them.     The    neurasthenic    appreciates    his   illness   keenly. 


PARESIS  363 

The  paretic  is  incapable  of  giving  a  connected  history  of  his  trouble. 
He  frequently  interrupts  his  speech  and  forgets  what  he  said  previously. 
When  reminded  he  does  not  attach  any  importance  to  it.  Frequently 
the  expression  of  his  face  does  not  correspond  to  the  subject  of  his  conver- 
sation: he  may  show  a  smile  while  speaking  of  death.  A  true  neurasthenic 
appreciates  keenly  his  trouble,  while  the  paretic  is  incapable  of  recog- 
nizing properly  the  ills  of  which  he  speaks.  In  a  true  neurasthenic 
mental  operations  are  readily  exhausted  but  not  disturbed  or  modified. 
The  paretic  presents  a  genuinely  diminished  intelligence.  Want  of 
power  of  criticism  and  of  judgment,  grave  omissions,  errors  in  daily 
acts,  change  of  moral  personality — are  all  met  with  in  paresis  but  not 
in  neurasthenia.  Besides,  the  cerebro-spinal  fluid  presents  certain  charac- 
teristics in  paresis  (see  page  375),  which  are  not  present  in  neurasthenia. 
Wassermann  test  on  blood  or  on  cerebro-spinal  fluid  is  positive  in  paresis, 
but  not  in  ordinary  neurasthenia. 

The  delirious  state,  the  confusional  state  of  the  paretics  may  be 
confounded  with  similar  states  occurring  in  intoxications.  These  are 
the  so-called  cases  of  pseudo-paresis  (alcoholic,  saturnine  and  others). 
If  the  diagnosis  is  difficult  at  the  beginninp,  a  subsequent  study  of  the 
cases  will  clear  it  up.  The  delirious,  confusional  states  are  only  transient 
and  the  obnubilation  of  intelligence  is  not  as  profound  in  intoxications 
as  in  paresis. 

The  alcoholic  subject  usually  suffers  from  hallucinations  of  a  terrifying 
nature  which  become  accentuated  at  night.  He  usually  appears  brutal 
and  stupid,  but  not  demented.  The  paretic  presents  a  reverse  condition. 
The  alcoholic  appears  sad  and  preoccupied,  the  paretic  indifferent  and 
apathetic.  If  an  alcoholic  develops  expansive  delusions,  they  have 
to  be  brought  out  and  can  be  ascertained  only  on  a  skillful  questioning 
of  the  patient.  The  paretic  does  it  unsolicited.  The  speech  of  the  pa- 
retic is  ataxic,  full  of  repetition  of  syllables  and  words.  The  speech 
of  the  alcoholic  is  merely  embarrassed,  thick  and  tremulous.  With- 
drawal of  intoxicants  is  followed  by  a  progressive  amelioration;  in  paresis 
the  symptoms  are  essentially  progressive.  Lymphocytosis  is  absent  and 
Wassermann  test  is  negative  in  alcoholism. 

Symptoms  of  lead  intoxication  (encephalopathy)  may  resemble 
paresis  especially  by  the  psychic  manifestations.  In  both  affections  a 
neurasthenic  state  and  mental  attitude  suggest  an  oncoming  dementia, 
but  in  encephalopathy  the  dementia  seems  to  dominate  the  entire  situa- 
tion from  the  onset.  The  physical  signs,  such  as  cachectic  appearance, 
difficulty  of  speech  of  a  gross  nature,  motor  disorder  of  paralytic  nature 
affecting  chiefly  the  extensor  groups  of  muscles,  abdominal  colic,  severe 


364  PARESIS 

headache,  the  blue  line  on  the  gums,  interstitial  nephritis,  arterio-sclerosis 
— are  all  found  in  lead  intoxication.  Finally,  the  regressive  evolution 
of  symptoms  after  an  appropriate  treatment  and  removal  from  the 
toxic  influence  is  characteristic  of  lead  intoxication. 

Lymphocytosis,  increase  of  albumen  in  the  cerebro-spinal  fluid, 
positive  Wassermann  reaction,  are  characteristic  of  paresis,  but  absent 
in  lead  encephalopathy. 

Diffuse  cerebro-spinal  syphilis  cannot  always  be  easily  differentiated 
from  paresis.  In  both  affections  a  neurasthenic  symptom-group  exists. 
Cerebro-spinal  syphilis  produces  a  profound  neurasthenic  state  which 
resembles  appreciably  paresis,  especially  in  the  initial  stage  of  the  latter. 
The  diagnostic  difficulty  is  especially  great,  when  in  both  affections 
Wassermann  and  Noguchi  reactions  are  positive  and  lymphocytosis  is 
present. 

In  cerebro-spinal  syphilis  the  onset  is  usually  acute,  in  paresis  slow 
and  insidious.  In  the  first  a  marked  diminution  of  intellectual  functions 
is  present  at  the  onset,  in  the  latter  the  intellectual  defect  is  less  marked 
at  first.  In  the  first  there  is  a  continuous  severe  headache  which  is 
worse  at  night,  in  the  latter  this  is  not  the  case.  In  the  first  there  is 
intellectual  inertia,  profound  apathy,  somnolence  and  a  special  dulness 
in  the  facial  expression.  Such  a  picture  is  not  seen  in  paresis.  There 
is  never  in  cerebro-spinal  syphilis  the  optimistic  carelessness  and  general 
exaltation  in  the  motor  and  sensory  spheres  which  are  observed  in  the 
expansive  form  of  paresis.  Alongside  of  the  apathy  and  loss  of  memory 
the  reasoning  power  may  be  totally  preserved  in  the  first,  but  this  is 
not  observed  in  paresis. 

Cerebro-spinal  syphilis  is  usually  accompanied  by  local  symptoms. 
Very  early  are  observed  ocular  changes,  such  as  optic  atrophy,  con- 
traction of  visual  fields,  palsy  of  other  cranial  nerves;  symptoms  of  spinal 
cord  involvement,  such  as  exaggerated  knee-jerks,  toe  phenomenon, 
ankle-clonus,  sensory  changes,  involvement  of  sphincters.  The  course 
of  cerebro-spinal  syphilis  is  characterized  by  great  variability  and  mobility 
of  the  symptoms  which  become  either  exaggerated  or  ameliorated.  In 
paresis  the  symptoms  are  invariably  progressive.  The  antisyphilitic 
treatment  has  a  remarkably  rapid  effect  on  the  symptoms  of  cerebro- 
spinal syphilis.  Such  a  course  of  events  is  not  observed  in  paresis.  The 
richness  of  symptoms  in  syphilis  is  such  that  it  is  possible  in  almost 
every  case  to  reveal  some  particular  feature  indicative  of  the  existence  of 
a  localized  focus  in  the  brain  and  spinal  cord.  The  incomplete,  unequal 
and  regressive  character  of  cerebro-spinal  syphilis  is  different  from  the 


PARESIS  365 

global  and  progressive  character  of  paresis.  Variability  and  capricious- 
ness  of  the  symptoms  are  typical  of  syphilis. 

Paresis  should  also  be  differentiated  from  tabes,  from  multiple  sclerosis, 
tumors  of  the  brain.  The  special  symptoms  of  these  affections  will  aid 
in  the  diagnosis. 

Etiology. — The  chief  determining  cause  is  syphilis.  It  is  not  in  the 
acute  stage  of  syphilis  that  paresis  develops,  but  years  after  the  initial 
infection  (from  five  to  fifteen  years).  Since  the  discovery  of  Wassermann's 
reaction  and  in  view  of  the  findings  of  Spirochsetae  in  paretic  brains  by 
Noguchi  and  Moore  (page  353),  paresis  has  proven  to  be  without  reserva- 
tion a  syphilitic  affection.  Plaut  (Allg. Zeist.f. Psych,  u.  Neur.,  1909) , for  ex- 
ample, obtained  in  every  one  of  his  cases  of  paresis  without  a  single  excep- 
tion a  positive  Wassermann  reaction.  In  a  number  of  cases  in  which  a 
history  of  syphilitic  infection  cannot  be  obtained,  the  Wasserman  test  is  of 
utmost  value.  While  formerly  such  cases  kept  us  in  doubt  as  to  syphilis 
being  the  only  cause  of  paresis,  now  with  Wasserman' s  discovery  all  hesita- 
tion must  be  set  aside.  Juvenile  paresis  showing  a  positive  Wassermann 
reaction  proves  that  congenital  syphilis  plays  also  a  powerful  role.  The 
following  peculiarities  concerning  the  relation  of  the  reaction  to  Paresis 
deserves  special  mention.  (1)  If  the  blood  serum  does  not  give  a  positive 
reaction,  the  cerebro-spinal  fluid  will  be  negative.  If  the  cerebro-spinal 
fluid  gives  a  positive  result,  the  serum  does  the  same.  (2)  If  the  serum 
gives  a  positive  result,  but  the  cerebro-spinal  fluid  a  negative,  the  disease  is 
not  paresis,  but  cerebro-spinal  syphilis.  (3)  The  reaction  of  the  cerebro- 
spinal fluid  is  more  important  in  paresis  than  that  of  blood  serum.  (4) 
Mercury,  iodides,  salvarsan  may  render  Wassermann  reaction  negative 
for  a  certain  time. 

The  predisposing  factors  are:  undue  and  prolonged  mental  efforts, 
worry  and  anxiety;  venereal  and  alcoholic  excesses;  traumata  of  the  head; 
sunstroke.  Men  are  more  frequently  affected  than  women.  The  age 
between  thirty  and  forty-five  is  the  most  favorite.  Juvenile  paresis  may 
occur  as  early  as  twelve  years  of  age.  As  to  the  countries,  the  higher 
the  degree  of  civilization,  the  larger  the  number  of  cases.  The  question 
of  neuropathic  hereditary  influences  is  not  entirely  settled.  However, 
not  infrequently  a  history  of  some  neurosis  or  insanity  is  traced  in  the 
antecedents  of  paretics. 

Treatment. — As  soon  as  paresis  is  recognized  or  even  suspected  in  its 
initial  period,  an  antisyphilitic  treatment  should  be  instituted  (see  Treat- 
ment of  Syphilis  of  the  Nervous  System) .  However,  in  spite  of  the  fact 
that  paresis  is  a  syphilitic  disease,  not  much  can  be  expected  from  the 
specific   medications.     The   value   of   Ehrlich's   new  remedy,    salvarsan, 


366  PARESIS 

has  been  sufficiently  tested  to  warrant  some  definite  conclusions.  If  it  does 
any  good  at  all,  it  improves  sometimes  the  general  health,  but  it  has  no 
effect  whatever  upon  the  course  of  paresis.  It  may  be  given  in  conjunc- 
tion with  mercury  and  iodides.  The  treatment  should  be  instituted  as 
early  as  possible.  However,  I  have  seen  favorable  results  in  prolonged 
treatment  with  iodides  given  progressively  in  very  large  doses  (300  or  500 
grains  daily).  In  view  of  the  fact  that  some  authors  observed  unfavor- 
able results  from  iodides,  the  increase  of  the  dose  should  be  made  very 
cautiously  and  upon  the  least  sign  of  intolerance  the  drug  should  be  dis- 
continued (see  also  Treatment  of  Tabes).  My  favorable  results  are  meant 
only  from  the  standpoint  of  some  acute  symptoms,  such  as  headache,  in- 
crease of  tremor,  insomnia,  disturbance  of  hearing  or  of  sight,  also  ataxia. 
It  is  worth  while  mentioning  the  fact  that  administration  of  salvarsan 
renders  the  Wassermann  reaction  negative.  In  spite  of  this  the  course  of 
paresis  is  not  altered. 

The  hygienic  and  dietetic  measures  are  more  important.  As  soon  as 
possible  a  paretic  should  be  removed  from  his  usual  occupations.  A  quiet 
life  (better  in  a  sanitarium  or  in  the  country),  avoidance  of  excitement 
and  worriment,  total  abstention  from  stimulants,  including  tea  and  coffee, 
regularity  in  meals  and  sleep,  a  diet  free  from  articles  which  are  likely  to 
produce  fermentation,  finally  hydrotherapy  (brief  douches,  baths,  etc.) 
are  all  extremely  beneficial.  Any  disturbance  of  the  gastro-intestinal 
tract,  of  renal  function,  of  sleep,  should  be  immediately  combated.  Sexual 
intercourse  must  be  avoided. 

If  this  plan  of  treatment  is  strictly  carried  out,  remissions  will  occur 
early  in  the  course  of  the  disease  and  if  a  remission  is  not  considered  a 
cure  (which  is  frequently  done  otherwise)  and  the  above  general  treat- 
ment kept  up  with  the  same  regularity  and  energy,  the  remissions  may  be 
prolonged  for  months  or   years. 

EARLY  PARESIS 

Early  Paresis. — The  recognition  of  paresis  in  its  earliest  stages  is  of 
utmost  importance  both  from  a  practical  and  medico-legal  standpoints. 
At  this  stage  the  symptoms  may  be  so  slight  that  they  are  easily  over- 
looked, proper  measures  are  neglected  and  deplorable  consequences  follow. 
The  patient  is  permitted  to  be  at  large,  to  travel,  to  transact  business, 
to  govern.  It  is  only  when  grave  errors  are  committed  that  attention  is 
drawn  to  the  patient's  condition. 

Psychic  Status. — The  most  delicate  and  the  highest  mental  functions 
are  invaded  the  earliest.  They  are  therefore  particularly  noticeable  in 
persons  of  culture.     It  is  in  the  fulfilment  of  fine  acts  necessitating  the 


PARESIS  367 

most  delicate  discrimination  that  lapses  and  omissions  are  observed. 
Memory  is  affected  early  in  the  disease.  It  is  slight  and  observed  only 
at  intervals  in  words  and  acts.  Soon  the  patient  shows  omissions  in  his 
daily  work;  his  writing  shows  lapses  and  repetition  in  letters,  syllables 
and  then  in  words.  The  amnesia  leads  to  neglect  in  his  own  appearance. 
Punctuality  of  habits  is  neglected  and  the  patient  becomes  indifferent. 
Usual  reservation  in  language  is  replaced  by  loquacity  and  unreasona- 
bleness. He  is  conscious  of  the  defect  of  his  memory  but  he  is  not  dis- 
turbed by  it  in  the  least.  He  does  not  try  to  avoid  errors.  He  even 
ridicules  his  own  mistakes.  This  indifference  toward  the  amnesia  is 
characteristic  of  early  paresis. 

At  the  same  time  changes  appear  in  the  character,  disposition  and 
moral  personality.  The  previously  cheerful  person  becomes  depressed, 
irritable,  apprehensive.  He  loses  interest  in  everybody;  is  neglectful  of 
his  duties,  of  his  home.  He  resembles  then  a  neurasthenic,  but  the 
resemblance  is  only  apparent  (see  differential  diagnosis). 

In  other  cases  a  totally  opposite  condition  is  observed.  The  usually 
conservative  man  has  become  exuberant  in  actions  and  spirits;  he  makes 
ambitious  schemes,  spends  money  in  an  unusual  way.  This  exaltation 
is  manifested  not  only  in  the  intellectual  and  motor  spheres,  but  also  in 
the  affective  sphere.  The  paretic  is  self-contented,  optimistic  and  he 
shows  a  tendency  to  excesses.  Immorality  is  the  natural  consequence; 
sexual  perversion  is  not  an  infrequent  phenomenon.  The  alterations  of 
the  moral  personality  occurs  often  long  before  the  intelligence  is  seriously 
affected. 

The  exuberance  is  only  superficial,  it  lacks  in  depth.  With  all  his 
agitation  the  early  paretic  is  not  capable  to  accomplish  much.  The 
contrast  between  what  he  appears  to  be  able  to  do  or  between  what  he 
promises  to  do  and  what  he  actually  does  is  striking.  He  is  easily 
fatigued.  He  is  always  ready  to  undertake  work,  but  is  unable  to  persist 
in  it. 

A  characteristic  feature  is  the  inability  of  the  patient  to  realize  his 
infirmity  in  spite  of  his  exuberant  spirit.  He  does  not  appreciate  the  fact 
that  he  cannot  continue  his  usual  occupation.  Another  interesting 
phenomenon  is  the  early  defect  of  criticism.  When  the  patient  is  con- 
fronted with  the  most  evident  manifestations  of  his  affection,  he  does 
not  pay  the  slightest  attention  and  rather  ridicules  them. 

To  sum  up,  defective  memory,  weakened  faculty  of  attention,  of 
judgment,  of  criticism,  perversion  of  moral  sense,  of  disposition,  radical 
change  in  conduct,  inversion  of  affective  faculties — all  constitute  character- 
istic psychic  manifestations  of  the  early  period  of  paresis. 


368  PARESIS 

Somatic  Symptoms.  Apoplectic  seizures,  epileptic  seizures,  attacks 
of  aphasia — are  the  three  groups  of  somatic  manifestations  that  not  in- 
frequently announce  the  oncoming  paresis. 

The  apoplexy  may  consist  only  of  sudden  mental  hebetude,  or  of  a 
genuine  loss  of  consciousness  followed  by  monoplegia,  hemiplegia  with  or 
without   aphasia.     These  phenomena  arc   usually  brief  and  temporary. 

Epileptic  seizures  are  mostly  of  petit  mal  form.  They  may  be  also  of 
grand  mal  form,  generalized  or  local.  In  the  latter  case  (local)  they  may 
be  of  the  sensory  variety:  sudden  paresthesias,  such  as  tingling  or  burning 
on  one  hand,  on  one  side  of  the  face. 

Aphasia  may  occur  without  hemiplegia.  The  attack  is  sudden  and 
transitory,  but  it  has  a  tendency  to  recur. 

Pupils.— In  fifty  out  of  fifty-nine  patients  studied  by  me  from  the 
standpoint  of  early  paresis  the  pupils  were  found  irregular  and  especially 
unequaL  Sluggishness  of  light  reflex  is  another  frequent  symptom. 
Ocular  palsies  are  not  very  infrequent,  but  they  are  usually  temporary. 

Reflexes. — The  knee-jerks  are  mostly  exaggerated.  Babinski's  sign  is 
rare.     Paradoxical  reflex  is  not  infrequent. 

Speech  and  tremor  are  both  rarely  altered  in  early  paresis. 

Early  paresis  presents  a  specially  important  chapter  from  the  medico- 
legal standpoint.  By  reason  of  gradually  developing  moral  and  affective 
perversions  which  announce  the  onset  of  the  disease,  the  initial  period  of 
paresis  is  the  real  medicolegal  period.  It  is  then  that  misdemeanors  are 
frequently  misinterpreted  and  that  the  individuals  are  sent  to  prison. 
Early  paretics  not  infrequently  marry.  The  new  life  the  patient  leads  in 
association  with  sexual  excesses  aggravates  the  condition.  Early  paretics 
are  able  to  carry  on  their  former  habits  and  other  acts  concerning  their 
usual  occupation,  but  they  do  it  automatically.  The  slight  lapses  of 
memory,  the  digression  in  their  conduct  apart  from  their  regular  work,  the 
changes  of  disposition  and  sentiments — are  usually  all  attributed  to  fatigue 
or  absent-mindedness.  They  are  permitted  to  have  liberty  of  action  and 
to  continue  in  responsible  positions  until  misconduct  of  grave  nature  and 
consequences  occurs.  It  is  at  this  period  that  illegal  acts  and  crimes  are 
most  frequently  committed.  Its  recognition  and  proper  interpretation 
is  a  matter  of  grave  importance.  Civil  capacity  and  legal  responsibility 
should  be  considered  as  having  no  existence  as  soon  as  the  earliest  signs 
of  paresis  begin  to  make  their  appearance. 


CHAPTER  XXIII 

LUMBAR   PUNCTURE  AND  CEREBRO -SPINAL  FLUID 

Lumbar  puncture  is  a  necessary  procedure  at  bedside.  It  gives  an 
opportunity  for  study  of  the  cerebro-spinal  fluid  besides  the  therapeutic 
effect  procured  by  extraction  of  a  certain  amount  of  the  latter  and  by 
injection  of  soluble  drugs  or  specific  sera  into  it. 

Anatomical  Points. — The  arachnoid  sac  which  does  not  contain  the 
spinal  cord,  but  contains  only  the  nerves  of  the  cauda-equina,  extends 
normally  from  the  second  lumbar  vertebra  to  the  second  sacral  .vertebra. 
It  is  between  these  two  extreme  points  that  a  puncture  of  the  sac  can  be 
made.  As.  in  children  especially,  the  cord  reaches  lower  down,  it  is  ad- 
visable in  every  case  not  to  puncture  between  the  second  and  third 
lumbar  vertebrae.  The  most  reliable  place  is  the  space  between  the  fourth 
and  fifth  lumbar  vertebrae.  A  transverse  line  drawn  between  the  crests  of 
iliac  bones  passes  exactly  at  the  level  of  the  spinous  process  of  fourth 
lumbar  vertebra.  Immediately  beneath  is  the  fourth  lumbar  space.  It 
is  there  that  the  puncture  is  made. 

Technic. — The  position  to  be  given  the  patient  is  variable:  some 
prefer  a  lateral  position,  others  prefer  a  sitting  position.  The  latter 
presents  certain  inconveniences,  namely  a  too  rapid  issue  of  the  fluid  or 
fatigue  of  the  patient.  When  the  patient  b'es  on  his  side,  he  is  placed  as 
near  as  possible  the  edge  of  the  bed;  the  head  is  slightly  raised  on  a  pillow, 
the  thighs  are  flexed  as  strongly  as  possible  over  the  pelvis  so  that 
a  marked  posterior  convexity  of  the  lumbar  spine  is  obtained  and  thus  a 
maximum  separation  of  the  two  vertebrae  is  produced.  The  skin  is  then 
washed  thoroughly  and  painted  with  tincture  of  iodin.  Rigorous  antiseptic 
precautions  must  be  observed  by  the  operator  with  regard  to  his  hands 
and  the  instrument  to  be  used.  Local  anesthesia  is  not  necessary.  The 
trocar  or  a  needle  of  9-10  cm.  in  length  and  0.8  mm.  in  diameter  must 
be  used.  It  must  be  fine  but  at  the  same  time  solid.  In  muscular  or 
insane  individuals  a  very  strong  needle  is  necessary.  The  operator  then 
places  the  index  of  the  left  hand  on  the  space  to  be  punctured.  The 
needle  is  then  seized  firmly  with  the  right  hand  and  plunged  at  a  half  of 
a  centimeter  laterally  to  the  place  outlined  with  the  finger  of  the  left  hand. 
Slowly  but  progressively  the  needle  is  advanced  toward  the  median  line 
and  slightly  upward.  At  a  distance  of  about  4  to  6  cm.  in  an  adult  and 
24  369 


370  LUMBAR    PUNCTURE 

of  i  to  3  cm.  in  a  child  the  needle  reaches  the  spinal  canal  and  per- 
forates the  arachnoid  sac.  The  cerebro-spinal  fluid  appears  imme- 
diately at  the  outer  end  of  the  needle.  When  the  desired  amount  is 
collected  in  a  sterile  tube,  the  needle  is  withdrawn  abruptly,  the  punc- 
ture of  the  skin  is  obliterated  with  collodion.  It  is  not  always  easy 
to  enter  the  spinal  canal.  Frequently  the  needle  strikes  against  the 
vertebrae;  the  needle  is  then  slightly  withdrawn  and  with  a  slight  devia- 
tion from  the  original  direction  will  eventually  penetrate  the  canal. 
Sometimes  it  is  necessary  to  withdraw  the  needle  entirely  and  renew 
the  operation.  It  may  happen  that  instead  of  cerebro-spinal  fluid  blood 
appears  at  the  outer  end  of  the  needle.  The  blood  frequently  rapidly 
disappears  and  clear  fluid  makes  its  appearance.  If  the  blood  persists, 
the  needle  must  be  withdrawn  and  a  new  puncture  made;  the  blood  was 
probably  due  to  a  puncture  of  a  small  intradural  vein.  Sometimes 
toward  the  end  of  the  operation  the  patient  is  seized  with  cramps  in  the 
thighs;  they  are  due  to  compression  of  some  nerve  filaments  of  the  cauda 
equina.     They  do  not  last  long. 

Accidents. — Lumbar  puncture  is,  generally  speaking,  an  innocuous 
operation.  However,  untoward  symptoms  have  been  observed.  Vertigo, 
headache,  nausea,  vomiting,  pain  in  the  back,  convulsions  may  occur, 
but  they  do  not  last  longer  than  a  few  hours.  Occasional  death  has  also 
been  observed  especially  in  cases  of  cerebral  tumors  and  when  a  too  large 
amount  of  cerebro-spinal  fluid  was  withdrawn.  Sicard  has  formulated 
the  following  precautions  to  be  observed  in  lumbar  punctures. 

(i)  Avoid  lumbar  puncture  in  brain  neoplasms  in  which  headache, 
nausea,  vertigo  increase  when  the  patient  is  in  dorsal  position. 

(2)  Before  the  puncture  keep  the  patient  in  bed  for  24  hours. 

(3)  Always  use  the  lateral  position  for  the  operation. 

(4)  After  the  puncture  keep  the  patient  in  dorsal  position  during  48 
hours  with  the  head  but  slightly  elevated. 

(5)  Except  in  special  cases,  withdraw  only  4  to  8  c.c.  of  the  fluid  without 
aspiration. 

(6)  Use  a  very  fine  needle  0.8  or  0.9  mm.  to  reduce  to  a  minimum  the 
meningeal  injury. 

In  cases  of  brain  tumor  observe  the  following  rules: 

(1)  Before  the  puncture,  observe  horizontal  position  in  bed,  the  head 
but  slightly  raised,  for  48  hours. 

(2)  Puncture  in  lateral  position,  the  head  slightly  lowered  (Trend- 
elenburg's position). 

(3)  After  the  puncture  keep  the  same  position  with  the  head  still 
lower  for  24  hours;  then  horizontal  position  for  48  hours. 


CEREBROSPINAL    FLUID  37 1 

CEREBRO-SPINAL  FLUID 

The  study  of  cerebro-spinal  fluid  offers  a  great  deal  of  information 
from  a  diagnostic  standpoint. 

Cerebro-spinal  fluid  closed  up  in  the  ventricles  and  in  the  subarach- 
noid spaces  is  secreted  by  the  choroid  plexuses. 

Physical  Properties. — Normally  the  fluid  is  perfectly  clear.  In 
pathological  conditions  it  is  generally  turbid,  purulent  or  lacteous,  or 
else  in  exceptional  cases  also  clear.  In  tubercular  conditions  it  is  floccu- 
lent  and  coagulates  in  large  lumps. 

The  color  may  change.  When  it  is  red,  intra-meningeal  hemorrhages 
are  to  be  suspected.  Whether  the  hemorrhage  is  of  cerebral,  bulbar  or 
meningeal  origin,  or  else  in  cases  of  contusions  or  fractures  of  the  skull, 
the  cerebro-spinal  fluid  will  present  either  a  frankly  hemorrhagic  color  or 
a  yellowish  or  greenish  discoloration.  Sometimes  however  the  bloody 
fluid  may  originate  from  the  tissues  through  which  the  needle  passes 
before  it  reaches  the  spinal  canal.  The  following  differential  features 
have  been  emphasized  by  Tufher. 

(i)  The  blood  from  an  accidental  cause  coagulates;  when  it  is  from  the 
cerebro-spinal  fluid  it  does  not  coagulate,  but  presents  a  sediment 
at  the  bottom  of  the  tube. 

(2)  If  the  bloody  fluid  is  centrifugated  and  presents  a  clear  aspect, 
the  hemorrhage  was  accidental  and  due  to  the  puncture  of  the  external 
tissues.  On  the  contrary,  if  the  fluid  remains  colored,  the  blood  existed 
in  the  subarachnoid  cavity. 

In  the  course  of  acute  cerebro-spinal  meningitis  the  cerebro-spinal 
fluid  may  be  of  yellowish  color.  The  latter  is  probably  due  to  small 
meningeal  hemorrhages. 

Tension. — In  cerebral  tumors,  in  meningitis,  in  syphilis  (secondary 
period) ,  in  epilepsy  there  is  increased  tension. 

Chemical  Properties. 

Reaction. — Normally  it  is  alkaline. 

Albumen. — In  normal  condition  albumen  is  either  absent  or  it  is 
present  in  extremely  small  quantities.  In  pathological  states  there  is 
increase  of  albumen.  In  Paresis,  Tabes,  in  Syphilis  of  the  Nervous  system 
albumen  content  is  increased.  The  majority  of  acute  states  present 
hyper-albuminosis,  such  as  tubercular  meningitis,  cerebro-spinal  menin- 
gitis, meningeal  symptoms  accompanying  infectious  diseases  and  intoxica- 
tions (lead).  When  the  quantity  of  albumen  in  above  0.25  or  0.30  grm. 
it  is  an  indication  of  meningeal  reaction. 


372  CEREBROSPINAL    FLUID 

Glucose. — Normally  there  is  0.50  gm.  of  sugar.  Diminished  quantity 
is  indicative  of  meningitis,  especially  cerebro-spinal  and  tubercular. 

Chlorids. — Normally  there  is  7  grm.  of  chloride  of  sodium.  Dimin- 
ished quantity  is  found  in  meningeal  conditions,  especially  in  the  tubercu- 
lar form. 

Cytological  characteristics. — In  normal  condition  the  cerebro-spinal 
fluid  contains  very  few  cellular  elements.  There  may  be  a  few  rare 
lymphocytes,  but  no  polynuclear  cells.  As  soon  as  the  meninges  are 
inflamed,  rapidly  a  lecocytosis  develops.  Lymphocytes  and  poly- 
nuclear are  the  two  forms  that  figure  prominently  in  pathological  condi- 
tions. Speaking  generally,  according  to  Sicard  polynucleosis  is  an  indica- 
tion of  a  grave  pathological  condition,  lymphocytosis  of  a  less  serious 
state  or  of  a  chronic  condition.  But  to  this  rule  there  are  many 
exceptions. 

Polynucleosis  is  observed  in  the  acute  stage  of  epidemic  cerebro- 
spinal meningitis,  sometimes  also  in  the  beginning  of  tubercular  meningitis. 
The  number  of  cells  is  in  relation  with  the  intensity  of  the  disease.  When 
the  latter  improves,  the  polynuclears  are  gradually  substituted  by  lympho- 
cytes. Polynucleosis  accompanies  meningeal  infections  of  a  strongly 
microbic  nature  (except  tubercular), viz.  epidemic  cerebro-spinal,  other 
forms  of  meningitis,  cranial  traumatism,  otitic  complications.  Tubercular 
meningitis  is  essentially  a  lymphocytic  condition. 

Syphilis  at  any  period  (except  the  chancre)  presents  essentially  a 
lymphocytosis.  In  acquired  senilis,  specific  meningitis  acute  or  chronic, 
specific  meningoencephalitis,  specific  neuritis,  also  in  hereditary  syphilis 
— lymphocytosis  is  characteristic.  In  Parasyphilitic  diseases,  tabes  and 
Paresis,  lymphocytosis  is  constant.  In  Paresis  during  the  paroxysms  a 
temporary  polynucleosis  is  observed.  The  same  lymphocytic  formula  is 
observed  in  acute  anterior  Poliomyelitis,  in  Landry's  paralysis,  in  Herpes 
Zoster,  in  Meningo-myelitis. 

Total  absence  of  leucocytic  reaction  is  observed  in  softening  and 
hemorrhage  of  the  brain,  cerebral  tumors,  syringomyelia,  disseminated 
sclerosis,  myopathies,  neuritis,  provided  there  is  no  syphilitic  factor. 

Chorea  presents  not  infrequently  lymphocytosis  because  not  infre- 
quently it  is  associated  with  a  meningeal  reaction. 

Bacteriology. — Microorganisms  may  be  discovered  in  the  cerebro- 
spinal fluid  either  by  direct  examination,  culture  or  inoculation.  Great 
varieties  of  microbes  have  been  found:  streptococcus,  pneumococcus, 
Eberth's,  Loffler's  and  Pfeiffer's  bacilli  and  coli-bacillus.  The  most  in- 
teresting ones  are:  meningococcus  of  Wechselbaum  and  tubercle  bacillus. 
The  first  is   the  specific  microorganism   of  the  epidemic  cerebro-spinal 


CEREBROSPINAL    FLUID  373 

meningitis,  the  second  of  tubercular  meningitis  (see  the  respective 
chapters) . 

Wassermann  Reaction. — In  1901  Bordet  and  Gengou  discovered  the 
fundamental  principle  known  as  "absorption  or  deviation  of  the  comple- 
ment." They  introduced  the  terms  antigen  and  antibodies  to  designate: 
the  former — a  substance  which  injected  into  an  animal  will  produce  an 
immune  serum;  the  other — an  antagonizing  substance  which  is  necessary 
for  the  immunizing  action  of  the  serum.  There  is  a  complement  in  every 
normal  serum  which  is  specific  for  each  animal.  Moreover  if  the  blood 
corpuscles  of  one  animal  are  injected  into  another  of  a  different  species, 
these  corpuscles  disappear  with  the  production  in  the  serum  of  a  specific 
hemolysin.  If  the  antibody  or  the  complement  is  removed,  the  specific 
hemolytic  action  of  the  serum  is  lost.  If  the  antibody  is  linked  up  to  the 
antigen  in  the  presence  of  the  complement,  both  the  antibody  and  comple- 
ment become  inactivated.  The  method  of  determining  if  the  blood  serum 
or  cerebro-spinal  fluid  contains  the  antigen  or  the  antibody  is  known 
under  the  name  of  deviation  of  the  complement.  The  determination  of 
the  presence  of  syphilitic  antibodies  and  antigen  constitutes  the  basis  of 
Wassermann's  test. 

Wassermann  reaction  is  regarded  at  present  one  of  the  most  valuable 
assets  in  medicine.  It  enables  to  decide  difficult  and  perplexing  diagnoses 
in  obscure  cases.  It  is  extremely  useful  in  Syphilis  and  Parasyphilis  of 
the  nervous  system.  It  is  the  consensus  of  opinion  that  when  it  is  posi- 
tive, it  means  syphilis,  but  when  the  reaction  is  negative,  it  does' not  ab- 
solutely exclude  syphilis,  as  some  factors  may  influence  the  reaction. 
There  is  at  a  present  sufficiently  large  number  of  facts  proving  that  in 
latent  tertiary  syphilis  the  Wassermann  reaction  is  the  more  rarely  observed 
the  more  energetic  is  the  treatment  with  mercury.  A  positive  reaction 
changes  into  a  negative  one  under  the  influence  of  mercury.  Bizzozero 
(Mediz.  Klinik,  1910)  made  investigations  with  reference  to  iodides.  In  a 
series  of  cases  he  observed  that  when  under  the  influence  of  iodides  the 
morbid  symptoms  improve,  the  positive  Wassermann  reaction  equally 
becomes  negative.  Nichols  and  Craig  (/.  Am.  Med.  Ass'n.,  191 1)  have 
shown  that  after  ingestion  of  considerable  amounts  of  alcohol  within 
twenty-four  hours  of  making  the  test  a  positive  serum  may  be  rendered 
negative,  and  in  some  cases  the  serum  will  strongly  give  a  negative  reac- 
tion for  as  long  as  three  days  after  the  administration  of  alcohol.  A 
careful  inquiry  therefore  should  always  be  made  regarding  the  recent  use 
of  alcohol  before  the  Wassermann  test  is  made. 

On  the  preceding  pages  the  importance  of  the  leucocytic  formula  in  the 
cerebro-spinal  fluid  was   strongly  emphasized.     It  is  interesting  to  ob- 


374  CEREBROSPINAL    FLUID 

serve  that  there  is  no  parallelism  between  the  leucocytic  reaction  and 
Wassermann  reaction.  The  first,  for  example,  may  be  very  marked  and 
the  Wassermann  test  negative  for  the  cerebro-spinal  fluid. 

Diagnostic  Significance  of  Four  Reactions  in  Organic  Nervous  Dis- 
eases.— Wassermann  reaction  in  blood  serum  and  cerebro-spinal  fluid,  also 
lymphocytosis  and  increase  of  albumen  contents  in  the  cerebro-spinal  fluid 
appear  to  have  an  important  value  for  differential  diagnosis.  Nonne 
(Deutsch.  Zeitschr.f.  Nerv.,  191 1)  has  recently  made  a  complete  study  of 
these  four  reactions  in  167  tabetics,  179  paretics,  ninety-seven  indi- 
viduals with  cerebro-spinal  syphilis,  sixty-eight  cases  of  multiple  sclerosis, 
thirty-eight  cases  of  brain  tumors,  fourteen  cases  of  spinal  tumors.  His 
important  conclusions  are  as  follows: 

I.  Lymphocytosis  and  increase  of  albumen  (Phase  1).  There  is 
almost  constant  presence  of  lymphocytosis  in  syphilis  and  parasyphilis ; 
it  is  less  abundant  in  parasyphilis  than  in  syphilis  of  the  nervous  system. 

In  a  few  isolated  cases  there  is  absence  of  lymphocytosis.  In  eleven 
cases  of  tabes  the  latter  was  absent;  four  of  these  cases  were  stationary, 
seven  in  incipient  period. 

Among  the  syphilitic  forms,  specific  hemiplegia  is  most  frequently 
without  lymphocytosis  and  without  increase  of  albumen.  The  spinal 
forms  of  syphilis  always  present  lymphocytosis  and  Phase  I. 

In  organic  nervous  diseases  other  than  of  syphilitic  or  parasyphilitic 
character  these  two  reactions  are  slight.  However,  in  two  cases  of  Mul- 
tiple Sclerosis  the  first  reaction  alone  was  present. 

In  brain  tumors  the  two  reactions  are  usually  absent  or  very  slight. 

In  tumors  of  the  cord  seven  times  out  of  fourteen  Phase  I  was 
present,  but  lymphocytosis  absent. 

In  syphilis  without  involvement  of  the  nervous  system  the  two  reac- 
tions are  observed  in  20  to  30  per  cent.  The  two  reactions  have  a  practi- 
cal bearing:  they  help  to  ascertain  whether  the  nervous  system  is  in- 
volved. In  neurasthenics,  alcoholics,  epileptics,  whether  they  are  syph- 
ilitic or  not,  the  amount  of  albumen  is  normal.  The  increase  of  al- 
bumen has  no  absolute  value  for  a  differential  diagnosis  of  syphilitic 
and  parasyphilitic  diseases  of  the  nervous  system,  but  it  permits  to 
differentiate  functional  from  organic  nervous  diseases.  Lymphocytosis 
has  no  absolute  value  for  differentiation  of  syphilitic  and  parasyphilitic 
affectious. 

II.  Wassermann  Reaction  with  Blood  Serum  and  Cerebro-spinal 
Fluid. — (a)  A  positive  serum  reaction  is,  with  very  rare  exceptions,  an 
indication  of  syphilis.  Nonne  found  it  in  60  to  70  per  cent,  of  tabetics,  in 
90  to  95  per  cent,  of  paretics,  in  80  per  cent,  of  syphilitic  arteritis.     He  be- 


CEREBROSPINAL   FLUID  375 

lieves  that  a  positive  serum  reaction  controls  the  prognosis  in  syphilitic 
individuals.  He  believes  that  a  negative  Wassermann  is  almost  a  certain 
indication  that  there  is  no  paresis.  The  degree  of  the  positive  Wasser- 
mann has  a  certain  significance.  A  very  strong  positive  reaction  is  more 
frequently  observed  in  paresis  than  in  tabes  or  cerebro-spinal  syphilis. 

(b)  A  positive  reaction  with  the  cerebro-spinal  fluid  is  observed  in 
100  per  cent,  of  paresis.  The  frequency  is  less  marked  in  tabes  and 
still  less  in  cerebro-spinal  syphilis.  The  test  with  cerebro-spinal  fluid 
is  more  important  in  paresis  than  with  blood  serum.  When  serum 
gives  a  positive  result,  but  the  cerebro-spinal  fluid  a  negative,  the  disease 
is  not  paresis. 

With  these  four  reactions  errors  of  diagnosis  may  be  easily  avoided. 

THERAPEUTIC    INDICATIONS    WITH    REGARD    TO    CEREBRO-SPINAL 

FLUID. 

A.  Lumbar  Puncture  and  Withdrawal  of  Fluid. — The  withdrawal 
of  cerebro-spinal  fluid,  which  should  not  be  above  20  to  30  c.c,  is  indicated 
in  the  following  conditions. 

In  congenital  Hydrocephalus  some  favorable  results  had  been  reported. 
Permanent  or  temporary  improvement  in  uremia  has  been  observed. 
In  tumors  of  the  cerebrum  or  cerebellum  relief  of  headache,  vomiting 
and  oedema  of  the  papilla  has  been  reported  by  very  competent  observers. 
However,  the  greatest  care  must  be  exercised  in  such  cases  and  only  very 
small  quantities  may  be  withdrawn,  as  rapid  decompression  may  produce 
a  fatal  syncope.  Epileptic  seisures  have  been  ameliorated  in  intensity 
and  frequency  from  occasional  withdrawal  of  small  amounts  of  cerebro- 
spinal fluid. 

The  main  indication  for  lumbar  puncture  is  in  Meningitis.  In  acute 
meningeal  conditions,  in  tubeicular  meningitis,  amelioration  of  symp- 
toms has  been  noticed.  The  most  favorable  results  have  been  obtained 
in  non- tubercular  forms,  cerebro-spinal,  serous,  purulent  and  syphilitic 
forms  of  meningitis. 

In  some  cases  of  serous  meningitis  excellent  results  have  been  obtained 
after  one  or  several  punctures.  In  Syphilitic  headache  good  results 
have  been  obtained.  Lumbar  puncture  has  a  distinct  therapeutic  value 
in  meningitis  of  bacterial  origin  but  non-tubercular. 

B.  Injections  of  therapeutic  agents  into  the  sub-arachnoid  cavity. 
The  sub-arachnoid  method  of  injection  is  superior  to  the  subcutaneous 

one  in  view  of  the  greater  absorbent  power  of  the  sub-arachnoid  cavity. 
This  fact  is  being  utilized  for  introduction  into  the  latter  of  drugs  and 
sera.     Antitetanic  serum,  antimeningococcus  serum — have  both  proven 


376  CEREBROSPINAL   FLUID 

to  be  of  great  utility.  Sulphate  of  magnesia  is  also  injected  into  the  sub- 
arachnoid cavity  in  cases  of  tetanus  with  satisfactory  results.  Cocain, 
eucain,  novocain,  stovain,  have  been  injected  for  analgesic  purposes; 
they  produce  a  rapid,  complete  and  sufficiently  durable  anaesthesia  to 
permit  operative  procedures  on  the  lower  extremities  and  some  abdominal 
viscera.  Certain  inconveniences  have  been  observed,  viz.  vertigo, 
headache,  nausea — of  which  all  may  last  several  days. 

Finally,  this  method  has  been  utilized  to  relieve  pain  occurring  in 
tabes,  sciatica,  lumbago  (see  these  Chapters).  Sicard  advised  the  use 
of  the  epidural  method  for  injection  of  analgesic  drugs.  This  method 
is  easier  and  is  not  followed  by  the  unpleasant  symptoms  of  the  first 
(see  for  details  page  443). 


CHAPTER  XXIV 

DISEASES  OF  THE  PERIPHERAL  NERVOUS  SYSTEM 

NEURITIS  (INFLAMMATION  OF  NERVES) 

Pathology. — An  inflammation  of  a  peripheral  nerve  may  be  confined 
to  the  connective  tissue  surrounding  the  nerve  (perineuritis)  or  lying  be- 
tween the  individual  bundles  of  the  nerves  (interstitial  neuritis)  or  else  to 
the  nerve  fiber  itself  (parenchymatous  neuritis).  In  the  majority  of 
cases  these  forms  are  combined. 


Fig.  119. — Neuritis  of  Sciatic  Nerve.     (Original.) 

Examination  shows  a  tumefaction  of  the  tissue  surrounding  the  nerve. 
Dilatation  of  the  blood  vessels,  extravasation  of  blood  and  sero-fibrinous 
exsudation  infiltrating  the  sheath,  leucocytes  around  the  vessels  are 
the  first  changes.  Gradually  these  alterations  extend  to  the  interstitial 
tissue  which  proliferates.  The  exsudation  within  the  sheath  produces 
pressure  on  the  nerve-fiber  itself.  In  the  latter  the  myelin  becomes 
broken  up  into  fine  particles.  When  the  disease  advances,  the  axis- 
cylinder  itself  takes  part  in  the  morbid  process.  It  undergoes  degener- 
ation and  in  more  pronounced  cases  it  atrophies  and  disappears.  In 
cases  of  primary  parenchymatous  neuritis,  viz.  without  the  involvement 

377 


378 


NEURITIS 


\, 


Inf.  Hcemorrhoidal 

of  Pudic 

Superficial  Perineal  of\ 

Pudic  and  Inferior 

Pudendal  of  small 

Sciatic 


Fig.  i 20. — Distribution  of  Sensory  Nerves  in  the  Skin.     {Flower.) 


NEURITIS 


379 


of  the  interstitial  tissue,  and  which  occurs  as  a  result  of  a  toxic  influence, 
there  is  an  exsudation  with  leucocytes  around  the  blood  vessels,  segmen- 
tation of  the  myelin  and  breaking  up  of  the  axis-cylinder,  swelling  of  the 
media  of  the  blood  vessels  and  proliferation  in  the  adventitia. 


S  1 

Fig.  121.  Fig.  122. 

Sensory  Radicular  Distribution.     {Bouchard  and  Brissaud.) 

Letters  C,  D,  L  and  S  indicate  respectively  cervical,  dorsal,  lumbar  and  sacral  roots. 

In  the  form  described  by  Gombault  under  the  name  of  periaxile 
segmentary  neuritis  only  some  segments  of  the  nerve-fibers  are  affected 
and  the  lesion  consists  of  fragmentation  of  the  myelin  sheath,  the  axis- 
cylnders  remaining  intact.  It  is  observed  in  toxic  or  infectious,  also 
traumatic  neuritis. 


38o 


NEURITIS 


Recovery  in  neuritis  corresponds  to  a  process  of  regeneration.  The 
latter  consists  of  longitudinal  division  and  sub-division  of  the  portions  of 
the  axis-cylinder  which  remained  intact  and  of  gradual  growth  of  the  new 
filaments  until  they  reach  the  tissues  innervated  by  the  nerve  before  its 
destruction;  at  this  stage  of  their  development  they  become  covered  by 
myelin. 


C8.D1 


L  1.2 


L3- 


Fig.  123. 
Letters  C,  D,  L  and  S  indicate  respectively  cervical,  dorsal,  lumbar  and  sacral  roots. 

Etiology. — Infections,  intoxications  and  local  factors  are  the  chief 
causes  of  peripheral  neuritis.  Microbes  or  their  toxins  in  infectious 
diseases  frequently  produce  a  multiple  neuritis,  but  they  may  also  be  the 
cause  of  a  localized  neuritis.     An  infected  wound  is  likely  to  set  up  an 


NEURITIS 


38l 


inflammation  of  a  nerve.  Trauma  sets  up  an  inflammation,  which  may 
involve  also  a  nerve.  An  inflammation  of  a  finger  may  extend  to  a  nerve 
or  nerves  of  the  arm  (ascending  neuritis) .  In  such  cases  the  neuritis  is  an 
upward  extension  of  an  inflammatory  or  septic  process  along  a  nerve  or 
nerves.     Suppurating  joint  or  acute  synovitis,  diseases  of  bones  (fracture, 


Fig.  124. 
Letters  C,  D,  L  and  5  indicate  respectively  cervical,  dorsal,  lumbar  and  sacral  roots. 

tumor,  caries),  dislocations  may  produce  a  neuritis  in  the  immediate 
neighborhood.  Diphtheria,  syphilis,  cancer,  tuberculosis  may  set  up  a 
toxic  neuritis.  Neuritis  due,  so  to  speak,  to  cold  is  often  of  toxic  or  infec- 
tious nature.  Intoxications  with  alcohol,  metals,  carbonic  acid,  may  pro- 
duce a  localized  neuritis.     In  the  course  of  diabetes,  gout,  chronic  rheu- 


382  NEURITIS 

matism,  not  infrequently  a  neuritis  (sciatica  for  example)  is  observed; 
autointoxication  is  probably  the  immediate  cause.  Finally  neuritis 
may  be  the  result  of  localized  ischemia  or  anemia  caused  by  partial  or 
complete  blocking  of  an  artery  such  as  embolus. 

Symptoms. — In  diseases  of  peripheral  nerves  sensory  disturbances 
are  very  conspicuous.  The  adjoining  plates  will  render  considerable 
assistance  in  the  study  of  various  sensory  areas  (Figs.  120,  121, 122,  123  and 
124).  The  main  sign  of  an  inflammation  of  a  nerve  is  pain.  It  is  usually 
intense  and  aggravated  by  movement,  or  anything  producing  a  congestion 
of  the  limb.  The  slightest  touch  or  pressure  upon  the  nerve  produces 
pain.  In  ascending  neuritis  there  is  an  excruciating  burning  pain  along 
the  nerve-trunk,  also  various  paresthesias,  as  formication,  numbness, 
etc.  The  objective  sensory  disturbances  in  the  area  of  distribution 
of  the  nerve  consists  at  first  of  hyperesthesia,  but  later  of  hypaesthe- 
sia  or  anaesthesia.  Sometimes  there  is  a  perverted  sensibility,  such  as 
cold  taken  for  heat  and  vice  versa.  Motor  disturbances  usually  ac- 
company the  sensory.  Twitching  and  tremor  are  present  at  the  begin- 
ning, but  when  the  condition  becomes  chronic,  paralysis  of  the  muscles 
innervated  by  the  affected  nerve  may  ensue.  Trophic  disturbances  of 
the  skin  are  frequent  (cedema,  cyanosis,  erythema).  Atrophy  of  the 
muscles  with  reactions  of  degeneration  is  very  frequent.  Sometimes 
the  muscular  atrophy  may  assume  the  type  Aran-Duchenne  (see  this 
chapter).  In  a  case  recently  reported  by  Long  (Nouv.  Iconogr.  de  Salpetr., 
19 1 2)  the  atrophy  first  appeared  in  the  left  hand,  three  years  later  in  the 
right  and  after  twelve  years  it  was  a  typical  Aran-Duchenne  form.  Mi- 
croscopically the  following  changes  were  found:  atrophy  of  a  large  number 
of  nerve-fibers,  proliferation  of  connective  tissue  between  the  fibers, 
hypertrophy  of  vasa  nervosum;  atrophy  of  muscular  fibers;  the  spinal 
cord  was  intact  except  a  few  isolated  cells  in  anterior  cornua  of  the 
cervical  enlargement  which  were  found  altered. 

A  neuritis  may  be  only  motor  or  sensory,  and  then  the  symptoms  are 
either  only  motor  or  only  sensory.  In  the  majority  of  cases  it  is  of  a 
mixed  type.  In  lead  intoxication  the  motor  phenomena  of  neuritis  are 
almost  exclusively  present.  In  syphilis  sensory  symptoms  alone  may 
be  present  for  a  long  time.  An  insolated  neuritis  of  a  sensory  filament 
of  a  nerve-trunk  was  reported  by  me  in  /.  of  Am.  Med.  Ass'n,  1909.  It 
was  a  record  of  four  patients  in  whom  the  lower  cutaneous  branch  of  the 
musculo-spinal  nerve  was  involved.  The  symptoms  were  exclusively 
sensory  (subjective  and  objective) ;  motor  and  trophic  disturbances 
were  absent. 

Course,  Termination,  Prognosis.- — Neuritis  usually  lasts  a  long  time — 


NEURITIS  383 

from  a  few  weeks  to  a  few  months;  it  may  also  last  years.  Its  prognosis 
is,  as  a  rule,  good.  It  depends  upon  the  cause.  Recoveries  are  frequent. 
Rapid  improvement  predicts  recovery.  Neuritis  of  rheumatism  and 
gout  usually  lasts  a  very  long  time.  As  a  rule  neuritis  of  long  duration 
presents  a  bad  prognosis  as  to  complete  recovery.  The  best  outlook  is 
in  neuritis  of  traumatic  origin. 

Diagnosis. — Marked  changes  in  objective  sensations,  especially  anaes- 
thesia over  the  area  of  distribution  of  the  nerve,  paralysis  with  atrophy  and 
reaction  of  degeneration,  are  the  chief  and  certain  symptoms  of  neuritis. 

Treatment. — Rest  of  the  affected  limb  is  the  first  indication.  Ideal 
rest  is  obtained  when  the  limb  is  immobilized  by  appropriate  means. 
This  procedure  gives  considerable  relief  from  pain.  In  addition  to 
immobilization  local  applications  of  extreme  cold  or  extreme  heat  are 
advisable.  A  very  good  procedure  is  to  place  the  affected  part  in  very  hot 
water  for  about  half  an  hour  or  even  an  hour,  two  or  three  times  a  day. 
In  two  cases  the  only  relief  could  be  obtained  from  a  continuous  immersion 
of  the  arm  in  hot  water.  Counter-irritation  by  fly-blister  and  sedative 
applications  may  be  useful  in  relieving  pain.  In  ascending  neuritis  the 
pain  may  be  so  intense  that  relief  can  be  obtained  only  by  opening  the 
wound  and  removing  the  cause  of  irritation;  if  the  latter  does  not  help, 
nerve  section  or  even  amputation  are  the  only  means.  Internally  coal- 
tar  products,  sedatives  should  be  given.  Morphine  should  be  resorted 
to  as  a  last  resort.  Massage  can  be  used  only  when  the  acute  symp- 
toms, and  especially  pain,  have  subsided.  Massage  and  electricity  are 
advisable  for  combating  the  atrophy. 

When  this  treatment  has  failed  and  the  pain  is  intense,  surgical  inter- 
vention, as  nerve-stretching  or  nerve-section,  is  indicated. 

Neuritis  of  Ischemic  Origin. — A  total  or  incomplete  obliteration 
of  a  blood  vessel  may  produce  irritation  and  cause  a  degeneration  of  a 
nerve  and  subsequently  a  paralysis.  In  such  cases  there  is  an  insufficient 
blood  supply  to  the  nerve  and  muscles  either  by  compression  (ligature, 
surgical  apparatus,  tumors,  enlarged  glands)  or  by  embolus,  thrombus, 
obliterative  arteritis.  Cases  of  this  kind  have  been  reported  by  Broca, 
Charcot,  Delherme,  Babinski,  Liston,  Mally,  Volkmann  and  others. 
The  symptoms  are:  paralysis  and  atrophy  with  the  reactions  of  degenera- 
tion. Pathologically  there  will  be  degeneration  of  the  nerve,  if  the 
ischemia  is  complete  and  durable.  In  incomplete  interruption  of  circula- 
tion the  paresis  of  the  limb  disappears  as  soon  as  the  obstacle  is  removed. 

Hypertrophic  Interstitial  Neuritis  of  Dejerine. — See  chapter  on 
Muscular  Atrophies. 


3§4 


NEURITIS 


NEUROMA 


Neuroma  tumors  may  be  isolated  and  single  or  multiple.  They 
usually  grow  in  the  sheath  of  a  nerve-trunk.  They  may  be  soft  (lipo- 
mata,  myxomata)  or  hard  (nbro-neuromata).  The  former  are  benign,  the 
latter  malignant.  The  first  are  usually  single,  they  cause  pain  along 
the  nerve,  they  are  tender  to  touch;  they  can  easily  be  removed. 

The  malignant  form  (fibro-sarcoma)  has  a 
special  predilection  for  the  sciatic  nerve.  It  has 
a  progressive  course  and  destroys  the  nerve,  pro- 
ducing besides  pain  also  paralysis  and  atrophy 
of  muscles.  Operation  is  the  only  remedy,  but 
the  prognosis  is  not  certain. 

Multiple  neuromata  are  fibrous  tumors  sit- 
uated either  in  the  skin  or  on  the  nerve- trunks. 
They  are  usually  diffuse.  They  are  known  under 
the  name  of  Recklinghausen's  disease.  When 
they  are  located  on  the  nerves  they  occupy  the  en- 
do-neurium;  degeneration  of  the  nerve-fibers 
follows.  Pain  is  present  and  paralysis  gradu- 
ally develops.  Not  infrequently  the  cranial 
nerves  are  the  seat  of  these  tumors.  When  the 
spinal  roots  are  affected,  the  tumors  may  be  found 
also  in  the  cord.  The  subcutaneous  tumors  are 
usually  soft  and  consist  of  plexiform  bunches  of 
nerve-fibers  described  by  Verneuil.  Other  symp- 
toms: (i)  pigmentation  of  the  skin  in  brownish 
patches  usually  situated  on  the  trunk  and  upper 
portions  of  the  limbs;  (2)  mental  manifestations, 
such  as  depression  and  diminution  of  intelligence. 
The  prognosis  is  uncertain  (Fig.  117). 

Neuroma  following  amputation  consists  of  a 
bulbous  mass  developing  on  the  proximal  end 
surface  of  a  divided  nerve.     It  is  due  to  a  rapid 

growth  of  the  sectional  axis-cylinders  and  proliferation  of  the  surround- 
ing connective  tissue.  It  is  usually  very  painful  to  pressure  and  thus  it 
prevents  from  using  an  artificial  limb.  Twitching  of  the  muscles  is  also 
observed  and  such  a  neuroma  may  be  the  point  of  departure  for  epileptic 
seizures.  Removal  of  such  tumors  is  a  necessity  but  recurrences  are. 
frequent. 


Fig  125. — Neurofi- 
bromatosis (v.  Reckling- 
hausen's disease.) 


MULTIPLE   NEURITIS 


385 


MULTIPLE  NEURITIS 

Pathology. — The  lesion  described  in  the  preceding  chapter  is  the 
same  when  several  nerves  are  simultaneously  affected.  The  special 
feature  of  polyneuritis  lies  in  the  fact  that  the  atrophic  state  of  the  nerves 
is  more  marked  than  the  inflammatory,  and  that  perineuritic  and  inter- 
stitial changes  are  extremely  slight,  while  the  nerve-fibers  are  markedly 
altered.  Another  peculiarity  is  found  in  changes  in  the  central  nervous 
system.  Thus  poliomyelitic  foci  in  the  cells  of  the  anterior  cornua,  de- 
generative condition  of  the  posterior  columns  (see  my  case  in  Amer. 
Medic,  1905),  inflammation   of   the  bulbar  nuclei,  have  been  observed 


Fig.  126. — Lead  Multiple  Neuritis,  Showing  Involvement  01  the  Posterior  Columns 

or  the  Cord.     {Original.) 


sometimes.  Evidently  the  chemical  poison  which  is  the  chief  cause  of 
multiple  neuritis  affects  almost  all  parts  of  the  nervous  system  but  to  a 
different  extent  in  various  cases:  sometimes  the  peripheral  nerves,  motor 
or  sensory  or  both,  at  another  time  the  nerves  and  the  cord. 

Etiology. — Intoxications  are  the  most  frequent  causes.  Alcohol  oc- 
cupies the  first  place.  Lead,  mercury,  arsenic,  phosphorus,  carbonic 
acid  gas,  intoxications  of  alimentary  origin  (ptomain)  are  also  frequent 
causes.  Infectious  diseases  play  a  great  role.  Typhoid  fever,  diph- 
theria, small-pox,  erysipelas,  pneumonia,  scarlet  fever,  grippe,  dysentery 
and  tuberculosis  are  not  infrequently  accompanied  or  followed  by  multi- 
ple neuritis.  In  these  cases  the  toxins  are  the  immediate  cause.  In  lep- 
rosy and  beriberi  the  neuritis  is  due  to  a  direct  action  of  microorganism. 

Syphilis,    malaria,    diabetes,    puerperal    state,    tobacco  and  arterio- 
sclerosis are  less  frequent  causes. 
25 


386  MULTIPLE   NEURITIS 

The  interstitial  hypertrophic  neuritis  of  Dejerine  and  Sottas  is  described 
in  a  separate  chapter. 

Symptoms. — Irrespective  of  their  causes  all  forms  of  polyneuritis 
present  a  common  clinical  picture,  which  will  be  described  here;  the  special 
symptoms  characteristic  of  each  variety  will  be  mentioned  later. 

General  Symptoms. — In  the  subacute  form  there  is  usually  noticed 
a  rapidly  progressing  loss  of  power  in  the  lower  extremities.  The  paraly- 
sis is  more  marked  in  the  lower  part  of  the  limb  than  in  the  upper.  When 
the  disease  advances,  the  upper  extremities  become  affected.  As  the  ex- 
tensors are  particularly  involved,  various  abnormal  attitudes  of  the  limbs 
are  acquired.  Wrist-drop  and  foot-drop  are  characteristic  of  polyneuritis. 
The  gait  is  typical:  being  unable  to  flex  the  foot,  the  patient  is  obliged  at 
each  step  to  flex  the  thigh  on  the  pelvis  in  an  exaggerated  manner.  This 
raising  of  the  thigh  with  the  toes  of  the  foot  hanging  down  is  analogous 
to  the  gait  of  horses.  It  is  called  "steppage  gait."  The  paralysis  is  flac- 
cid. While  it  affects  most  frequently  the  extensor  muscles,  it  may  never- 
theless attack  any  muscle  or  group  of  muscles.  The  tendon  reflexes  are 
abolished  or  markedly  diminished. 

Gradually  the  muscles  of  the  thorax  and  abdomen  become  affected 
and  when  the  bulbar  nerves  are  invaded  the  condition  is  alarming. 

The  electrical  reactions  of  the  paralyzed  muscles  present  the  following 
changes.  At  first  there  is  a  diminution  of  response  to  f aradic  and  galvanic 
currents.  Later  on  the  faradic  contractility  is  abolished  and  reactions  of 
degeneration  are  manifest. 

Muscular  atrophy  follows  the  paralysis,  and  its  beginning  corresponds 
to  a  destruction  of  the  axis-cylinders  of  the  nerves  distributed  in  the  mus- 
cles. It  is  very  frequent  in  polyneuritis,  and  it  may  make  its  appearance 
at  any  stage  of  development  of  the  paralytic  symptoms. 

Sensory  disturbances  are  very  common.  They  consist  of  all  sorts  of 
abnormal  sensations,  as  tingling,  burning,  numbness,  etc.;  of  spontaneous 
pain  or  of  pain  produced  by  the  slightest  pressure  or  movements  of  the 
limb;  of  objective  modifications  of  all  forms  of  sensations  (hypassthesia, 
anaesthenia,  hypalgesia,  analgesia) ;  of  loss  of  muscular  sense  (patient  is 
unable  to  appreciate  the  position  of  the  affected  limbs). 

Vaso  -motor  disturbances  are  occasionally  observed,  especially  in  poly- 
neuritis of  external  origin  (traumatism,  compression,  tumors).  They  are 
ulcers,  eruptions,  oedema,  hyperhidrosis  of  hands  and  feet. 

The  sphincters  are  usually  intact. 

In  the  course  of  multiple  neuritis  paralysis  of  cranialnerves  may  occur. 
The  oculo-motor,  the  abducens,  the  optic,  the  facial,  the  pneumogastric 
are  sometimes  involved.     Strabismus^  diplopia  loss  of  light  reflex,  optic 


MULTIPLE   NEURITIS 


387 


neuritis,   amaurosis,   facial  palsy,  paralysis  of  the  vocal  cords,   tachy- 
cardia, difficulty  of  respiration — are  all  observed  occasionally. 

The  acute  form  of  multiple  neuritis  differs  somewhat  from  the  preced- 
ing form.  The  onset  is  here  sudden,  accompanied  by  chills,  fever  and 
headache.  Rapidly  the  symp- 
toms develop.  Paralysis,  loss 
of  reflexes,  objective  sensory 
disturbances,  etc.,  appear  in 
the  same  succession  as  in  the 
previous  form. 

Course,     Termination, 
Prognosis. — In  the  sub -acute 
form  recovery  follows  the  ma- 
jority of  cases.     The  paralysis, 
the  atrophy,  the  sensory  dis- 
turbances gradually  improve, 
the  electrical  reactions  become 
normal.     In   some    cases    the 
paralysis  persists,  retraction  of 
tendons  and  immobilization  of 
joints  take  place  and  the  pa- 
tient is   permanently    crippled.     In    exceptional    cases    the   respiratory 
muscles  are  paralyzed  and  death  may  ensue  from  pulmonary  complica- 
tions.    The  acute  form  (not  to  be  confounded  with  Landry's  type  of 
polyneuritis)  bears  practically  the  same  prognosis  as  the  subacute. 


Fig.  127. 
Double  Wrtst-drop  in  a  Case  of  Lead  Palsy. 


Special  Features 

A.  Alcoholic  Polyneuritis. — This  is  the  most  frequent  of  all  forms  of 
multiple  neuritis  and  more  in  women  than  in  men.  The  disturbances 
are  both  motor  and  sensory.  The  latter  are  very  conspicuous;  they  may 
be  pronounced  while  the  motor  power  is  only  slightly  disturbed.  The 
objective  and  especially  the  subjective  sensory  disorders  described  in  the 
preceding  chapter  are  particularly  well  developed.  The  patient  complains 
early  of  unusually  severe  pain,  which  may  be  lancinating,  tearing  or  pull- 
ing. The  least  pressure  shows  exquisite  tenderness.  The  pain  leads  to' 
insomnia,  to  mental  depression. 

Cramps  in  the  calves  of  the  legs,  muscular  twitchings  occur  frequently. 
Intestinal  or  gastric  pain  occurring  in  paroxysms  is  common.  Muscular 
hyperaesthesia  is  particularly  marked;  the  least  pressure,  even  the  contact 
of  bed-clothes  is  intolerable.  Cutaneous  anaesthesia  in  the  limbs  is 
usually  only  in  their  distal  portions. 


388  MULTIPLE   NEURITIS 

The  paralysis,  gait,  reflexes  are  as  described  in  the  general  sympto- 
matology. However  in  alcoholic  neuritis  the  bilateral  paralysis  of 
the  extremities  has  a  tendency  to  generalization ;  it  gains  the  proximal 
ends  of  the  limbs,  the  thorax,  the  abdomen.  The  muscular  atrophy  is 
pronounced. 

The  paralysis  and  atrophy  produce  deformities  of  the  limbs.  When 
the  muscles  of  the  back  are  involved,  there  is  lordosis.  When  the  glutei 
are  involved,  the  patient  is  unable  to  rise  from  his  seat.  In  paralysis  of 
the  abdominal  muscles  the  expelling  power  of  the  bladder  and  rectum  is 
weakened.  If  the  muscles  of  the  neck  are  affected,  the  head  cannot  be 
moved  voluntarily.  Involvement  of  the  intercostal  muscles  interferes 
with  respiration.  Very  rarely  the  muscles  of  the  face  are  involved. 
Tremor  of  the  tongue  and  weakness  of  the  muscles  participating  in  articu- 
lation of  words  may  give  the  impression  of  paresis. 

Among  the  vaso  -motor  disturbances  oedema  of  the  lower  limbs  is  very 
common.     Profuse  sweating  is  frequent. 

Ocular  disturbances  are  frequent.  Retro-bulb ar  optic  neuritis, 
central  scotomata,  strabismus,  ptosis  and  sluggish  pupillary  reflex  to 
light — are  all  met  with  in  alcoholic  polyneuritis. 

Finally  psychic  disturbances  are  sometimes  observed.  Besides 
various  manifestations  which  are  usually  encountered  in  alcoholism,  such 
as  delirium,  confusion,  stupor  and  hallucinations,  there  is  a  special 
symptom-group  which  sometimes  accompanies  polyneuritis.  This  is  the 
so-called  Korsakoff's  psychosis.  It  consists  chiefly  of  confusion  with 
illusions  of  identity,  loss  of  orientation,  of  memory.  Delirium  and 
hallucinations  may  also  be  present. 

The  amnesia  covers  the  period  for  recent  events  and  for  the  time  of  the 
illness.  The  various  lapses  of  memory  are  filled  by  the  patient  with  a  great 
variety  of  fabrications  or  confabulations.  The  patient  described  events 
and  occurrences  which  never  took  place  and  does  it  with  minutest  details. 

The  amnesia  for  recent  events  is  considerably  facilitated  by  an 
inability  to  associate  or  synthetize  new  facts.  The  disturbance  in  associa- 
tion of  ideas  is  limited  to  new  perceptions  and  conceptions.  The  vol- 
untary psychic  activity  is  diminished,  but  the  patient  preserves  the 
old  intellectual  acquisitions  and  his  judgment  on  those  old  events  is 
intact. 

The  affection  lasts  from  a  few  weeks  to  several  years.  It  terminates 
either  in  death,  or  in  a  more  or  less  complete  recovery,  or  else  in  dementia. 

The  course  of  alcoholic  polyneuritis  is  usually  subacute,  lasts  several 
months  or  even  longer  and  in  a  large  number  of  cases  results  in  recovery. 
In  some  cases  the  recovery  is  incomplete:  deformities  of  the  extremities 


MULTIPLE    NEURITIS  389 

remain  permanent.  Pulmonary  tuberculosis  is  not  infrequently  the 
cause  of  death  in  the  protracted  cases.  The  prognosis  is  serious,  as  bulbar 
symptoms  may  develop  at  any  time  and  hasten  death  and  on  the  other 
hand  deformities  render  the  patient  powerless.  The  acute  cases  have 
ordinarily  an  unfavorable  prognosis.  In  a  case  that  I  had  under  my 
observation  for  eight  months  there  were  besides  the  involvement  of  the 
limbs  also  bulbar  symptoms  (difficulty  of  deglutition  and  of  respiration, 
tachycardia)  and  mental  disturbances;  the  patient  nevertheless  made  a 
perfect  recovery.  Recurrences  of  attacks  is  another  special  feature  of  the 
alcoholic  multiple  neuritis. 

B.  Lead  Neuritis  (Lead  Palsy). — Pathologically  it  presents  the'periaxile 
segmentary  form  of  Gombault  (see  Pathology).  The  paralysis  presents 
a  strictly  localized  character.  The  upper  extremities  are  the  seat  of 
predilection  for  lead  palsy.  The  latter  is  bilateral  and  symmetrical.  It 
may  present  the  antribrachial  type,  Aran-Duchenne's  type,  also  the 
scapulo-humeral  type  (see  Muscular  atrophies,  also  my  contribution  in 
New  York  Med.  Jour.,  1906).  The  first  is  the  most  common.  Gradually 
and  insidiously  the  extensors  of  the  fingers,  except  the  long  supinator 
and  the  long  abductor  of  the  thumb,  are  paralyzed.  This  leads  to  a 
special  attitude  of  the  hand.  The  latter  is  in  pronation  and  flexion  (wrist- 
drop), also  the  fingers  are  slightly  flexed. 

Muscular  atrophy  with  reactions  of  degeneration,  also  loss  of  1  en  exes 
develop  quite  early.     Cyanosis  of  the  hands  is  frequent. 

Sensory  disturbances  are  usually  absent.  In  some  cases  the  onset  of 
paralysis  is  preceded  by  some  pain  cr  parassthesia. 

Optic  neuritis,  amblyopia,  contraction  of  the  visual  field  are  sometimes 
observed. 

In  rare  cases  lead  palsy  may  become  generalized.  It  may  invade  the 
lower  extremities  and  even  the  trunk. 

When  the  lower  extremities  are  affected,  the  peroneal  muscles  are 
paralyzed,  but  the  tibialis  anticus  is  usually  spared. 

In  acute  cases  of  lead  intoxication  the  paralysis  may  become  general- 
ized. Paralysis  of  the  diaphragm  and  intercostal  muscles  causes  dyspnoea 
and  the  condition  becomes  alarming.  Ordinarily  the  disease  has  a  chronic 
course.  In  a  large  majority  of  cases  the  symptoms  improve  and  even  dis- 
appear completely.  Recurrences  are  not  infrequent.  The  prognosis  of 
lead  neuritis  is  as  a  rule  favorable,  but  the  damage  done  by  lead  on  other 
organs  and  tissues  may  be  so  great  that  life  is  in  danger,  as  for  example, 
in  cases  with  renal  complications.  In  making  the  diagnosis,  some  aid  may 
be  obtained  from  other  symptoms  of  lead  poisoning,  such  as  the  blue  line 
on  the  gums,  history  cf  colic,  encephalopathy,  tremor.     The  distinction 


390  MULTIPLE   NEURITIS 

between  wrist-drop  from  lead  neuritis  and  that  from  musculo-spiral  palsy  lies 
in  the  retention  of  power  of  the  long  supinator  muscle  in  the  first  affection. 
Schoenfeld  (Mediz.  Klinik,  May,  1913)  has  recently  called  attention 
to  the  importance  of  examination  of  blood  in  lead  poisoning.  He  points 
out  as  a  characteristic  finding  the  basophil  granulation  in  the  red  cor- 
puscles and  the  change  in  tint  of  these  basophil  substances  under  the 
action  of  certain  stains.  These  blood  findings  are  also  useful  to  control 
the  progress  toward  recovery;  in  18  of  40  patients  thus  kept  under  ob- 
servation the  blood  returned  to  normal  after  the  patients  had  changed 
their  occupation.     Negative  findings  exclude  lead  intoxication. 

C.  Arsenical  Neuritis  (Arsenical  Paralysis). — This  form  is  a  mixed 
type  of  neuritis :  motor  and  sensory.  It  affects  the  four  extremities,  the 
lower  before  the  upper,  and  while  the  extensors  are  particularly  involved, 
the  paralysis  affects  also  the  flexor  muscles  (unlike  alcoholic  and  lead 
palsies).  Ataxia  is  frequently  observed,  and  this  in  addition  to  the  loss 
of  reflexes,  gives  the  impression  of  tabes  (arsenical  pseudotabes  of  Dana). 
Psychic  disturbances  are  sometimes  observed.  Muscular  atrophy  with 
reactions  of  degeneration,  sensory  and  trophic  disturbances  are  identical 
with  those  of  other  forms  of  polyneuritis.  Subjective  sensory  disturb- 
ances are  usually  conspicuous  and  persistent.  They  are:  severe  tearing 
pain  in  the  limbs,  burning  in  the  toes  and  soles  of  the  feet.  The  skin 
and  muscles  are  very  tender  to  pressure.  This  hyperalgesia  is  frequently 
associated  with  anaesthesia  to  touch,  pain  and  temperature.  The  atrophic 
paralysis  may  spread  and  involve  not  only  the  proximal  ends  of  the  limbs, 
but  also  the  diaphragm  (the  intercostal  muscles  usually  escape) .  Tremors 
and  spasmodic  contractions  have  been  observed.  Loss  of  vibratory  sense 
or  osseous  sensibility  in  the  lower  extremities  was  observed  by  Byrnes 
(/.  Amer.  Med.  Ass'n.,  1909).  Among  other  symptoms  of  arsenical 
neuritis  may  be  mentioned:  pigmentation  of  the  skin,  vesicular  eruption, 
erythematous  and  cedematous  condition  of  the  soles  and  of  the  palms. 
Epileptiform  convulsions  have  occasionally  been  observed.  The  prog- 
nosis in  arsenical  neuritis  is  usually  favorable.  In  1900  an  epidemic  of 
arsenical  neuritis  occurred  in  Manchester  in  beer  drinkers.  The  analysis 
of  the  beer  discovered  the  presence  of  arsenic.  The  arsenic  was  derived 
from  sulphuric  acid  employed  in  preparation  of  glucose  used  in  brewing 
cheap  beer. 

Arsenical  neuritis  may  occur  from  medicinal  use  of  arsenic.  Thus  it 
was  observed  in  patients  suffering  from  chorea  who  used  arsenic. 

D.  Diphtheritic  Neuritis  (Diphtheritic  Paralysis).— It  usually  occurs 
during  convalescence  from  diphtheria.  It  affects  first  the  palate  and 
develops  slowly.     At  the  beginning  there  is  only  some  disturbance  in 


MULTIPLE   NEURITIS  39 1 

phonation  and  deglutition,  but  gradually  the  difficulty  becomes  greater. 
The  palatine  velum  hangs  down  and  does  not  contract  during  phonation. 
The  mucous  membrane  is  anaesthetic.  The  food  passes  through  the  nose. 
As  the  paralysis  extends  to  the  pharynx  and  larynx,  the  epiglottis  does 
not  close  the  larynx;  food  penetrates  into  the  respiratory  passages  and 
produces  pneumonia  or  else  direct  asphyxiation.  This  is  also  aided  by 
anaesthesia  of  the  larynx.  The  phonation  is  nasal,  labial  letters  are 
pronounced  indistinctly.  When  the  paralysis  reaches  the  recurrent 
laryngeal  nerve,  hoarseness  and  aphonia  will  be  the  result.  The  eye 
muscles  are  infrequently  involved.  Strabismus  with  diplopia,  ptosis, 
disturbed  pupillary  reflexes  (paralysis  of  accommodation  with  preserva- 
tion of  light  reflex),  amblyopia  are  all  observed. 

When  the  paralysis  shows  a  tendency  to  generalization,  the  lower 
extremities  become  affected  after  the  palate,  pharynx,  larynx  and  eyes. 
Like  in  the  preceding  forms  the  antero-external  group  of  the  leg  muscles 
are  paralyzed.  Steppage  gait  with  foot-drop  is  present.  Ataxia,  loss 
of  muscular  sense,  loss  of  reflexes,  intense  pain  gives  the  impression  of 
tabes  (pseudo-tabes).  The  muscles  of  the  upper  extremities,  also  of  the 
trunk,  may  follow  those  of  the  lower.  When  atrophy  develops,  it  is 
usually  rapid  and  reactions  of  degeneration  are  present. 

The  muscles  of  the  tongue,  lips  and  face  may  be  affected.  When  the 
pneumogastric  and  the  phrenic  nerves  are  involved,  cardio-pulmonary 
disturbances  are  present. 

The  course  of  the  disease  depends  upon  its  intensity  and  localization 
of  the  poison.  When  bulbar  symptoms  are  present,  death  usually  ensues. 
Dyspnoea,  syncope,  asphyxia,  aspiration  pneumonia  are  all  the  causes 
of  death.  Generally  speaking  the  prognosis  is  favorable,  especially 
when  the  paralysis  is  confined  to  the  palate.  In  the  latter  case  the  dura- 
tion is  about  two  to  three  weeks.  When  the  paralysis  is  generalized,  it 
lasts  several  months.  The  special  feature  of  diphtheritic  palsies  lies  in 
rapid  development  and  the  instability  of  the  motor  symptoms,  which 
disappear  in  one  place  to  appear  in  another  and  then  again  reappear  in 
the  first. 

E.  Carbonic  Gas  Neuritis. — In  cases  of  intoxication  with  carbon 
dioxide  the  following  special  symptoms  are  present:  anaesthesia  of  the 
extremities;  paralysis  at  first  of  antero-lateral  muscles  of  the  legs  and 
later  of  the  muscles  of  the  upper  extremities;  preservation  or  increase 
of  tendon  reflexes;  trophic  and  vaso-motor  disturbances;  mental  dis- 
turbances (particularly  amnesia).  Recovery  follows  almost  in  every 
case.  Mental  feebleness  and  anaesthesia  remain  a  long  time,  even  in 
the  most  favorable  cases. 


39 2  MULTIPLE    NEURITIS 

F.  Mercurial  Neuritis  is  manifested  by  paresis  or  paralysis  of  the 
antero-external  groups  of  muscles  of  the  legs  and  sometimes  also  of  the 
upper  extremities,  by  anaesthesia  in  certain  areas  and  hyperesthesia 
in  others,  finally  by  enfeeblement  of  the  mental  faculties. 

G.  Puerperal  Neuritis  may  occur  either  during  pregnancy  or  after 
labor.  In  the  first  case  usually  the  nerves  of  the  lower  limbs  are  involved, 
in  the  second  case  the  nerves  of  the  upper  limbs  are  affected.  Here  the 
neuritis  presents  certain  peculiarities  in  its  localization.  It  affects 
especially  the  terminal  branches  of  the  median  and  ulnar  nerves,  and 
mostly  on  the  right  side.  Sometimes  it  becomes  generalized  and  then 
involves  the  lower  limbs.  Sensory  disturbances  are  slight.  The  disease 
is  probably  infectious  in  nature  in  cases  which  occur  after  labor.  In 
neuritis  occurring  during  pregnancy  the  etiological  factor  is  probably 
disturbed  metabolism.     It  usually  ends  in  recovery. 

H.  Beriberi  or  Kakke. — Under  this  name  is  known  a  form  of  endemic 
or  epidemic  multiple  neuritis,  considered  infectious  in  origin,  and  met 
with  in  South  America,  Japan,  Philippines,  China.  Its  microorganism 
has  not  yet  been  discovered.  It  is  supposed  to  enter  the  body  thiough 
the  gastro-intestinal  tract.  The  disease  has  been  observed  in  connection 
with  prolonged  consumption  of  mouldy. rice. 

A  very  recent  experimental  study  by  Strong  and  Crowell  {Philippine 
Jour,  of  Science,  1912)  shows  that  there  is  no  evidence  which  could  suggest 
that  beriberi  is  an  infectious  disease,  that  beriberi  in  the  Philippine  Islands 
is  due  to  a  prolonged  consumption  of  a  diet  which  lacks  certain  substances 
necessary  for  the  normal  physiologic  needs  of  the  human  body.  For 
prevention  and  cure  of  beriberi  it  is  necessary  that  man  shall  be  supplied 
with  a  liberal  nutritious  diet  suitable  to  the  physiologic  needs  of  the 
body.  As  to  the  question  of  rice,  although  a  rice  containing  0.4  per  cent, 
of  phosphorus  will  prevent  the  appearance  of  polyneuritis  in  fowls, 
nevertheless  from  these  two  authors'  experiments  it  is  evident  that 
beriberi  in  man  may  be  produced  by  rice  containing  0.37  per  cent,  of 
phosphorus  pentoxide.  The  higher  the  phosphorus  content  of  rice  the 
less  is  the  liability  of  that  rice  to  produce  beriberi. 

The  following  are  the  chief  symptoms  of  the  disease.  As  an  early 
manifestation  must  be  mentioned  a  cardiac  syndrome  consisting  of  smallness 
and  rapidity  of  the  pulse,  dilatation  of  the  heart,  systolic  murmur.  Attacks 
of  cardiac  insufficiency  are  not  rare  (Edema  is  another  frequent  early 
symptom.  It  affects  the  lower  extremities,  abdomen,  neck  and  face,  also 
the  viscera;  pericarditis  is  not  rare;  increase  of  cerebro-spinal  fluid  in  the 
spinal  canal  has  also  been  observed.  Albuminuria  is  usually  absent. 
Absence  of  fever  is  the  rule.      The  nervous  symptoms  which  usually 


MULTIPLE    NEURITIS  393 

develop  after  an  acute  onset  consisting  of  nausea,  vomiting,  oppression,  are: 

(1)  paralysis  of  the  lower  extremities  and  especially  of  the  antero-external 
group  of  muscles;  the  gait,  reflexes,  attitude  are  same  as  in  other  forms 
of  polyneuritis.     The  upper  extremities,  thorax,  may  also  become  involved, 

(2)  anaesthesia  cf  the  affected  limbs  and  spontaneous  pain,  (3)  muscular 
atrophy,  (4)  mental  disturbances  similar  to  those  of  alcoholic  polyneuritis, 
(5)  respiratory  disturbances.  The  latter  symptom,  also  the  above-men- 
tioned cardiac  condition,  are  due  to  the  involvement  of  the  vagus  and 
phrenic  nerves.  Rapid  development  of  cardiac  and  pulmonary  disturb- 
ances is  characteristic. 

Beriberi  has  been  observed  under  three  varieties:  (1)  the  humid  or 
hydropic  form  in  which  the  oedema  is  most  conspicuous;  (2)  the  dry  or 
paralytic  form  in  which  there  is  no  oedema  but  paralysis  of  the  muscles 
is  the  most  prominent  symptom;  (3)  mixed  form  in  which  the 
symptomatology  is  complete.  In  all  forms  of  the  disease  the  cardiac 
symptoms  are  invariably  present.  The  duration  of  the  disease  is 
from  a  few  weeks  to  several  months. 

The  prognosis  is  grave,  although  recovery  is  possible. 

Death  usually  occurs  from  the  cardiac  failure  or  from  some  inter- 
current disease.  The  treatment  does  not  differ  from  that  of  other  forms 
of  multiple  neuritis.  Special  attention,  however,  should  be  given  to  the 
condition  of  the  heart.  Lumbar  puncture  may  be  useful  in  view  of  the 
increased  tension  of  the  cerebro-spinal  fluid. 

I.  Lepra  Neuritis  (Nerve  Leprosy). — In  this  form  of  leprosy  the 
changes  are  found  almost  exclusively  in  the  peripheral  nerve  trunks  and 
their  cutaneous  branches.  Changes  in  the  central  nervous  system,  if 
present,  are  secondary  to  the  general  leprous  infection.  Thickening  of 
the  nerves  (and  sometimes  nodules)  is  very  frequent  in  the  early  period 
of  the  disease,  but  in  the  advanced  stage  atrophy  is  usual.  The  leprosy 
bacillus  is  found  in  the  connective  tissue  between  and  around  the  nerve- 
fibers.  This  tissue  proliferates  and  through  pressure  on  the  nerve-fiber 
itself  produces  its  atrophy  (Figs.  128  and  129). 

Clinically  trophic  disturbances  are  observed.  The  joints  of  the 
phalanges  are  commonly  affected.  Ulcerations  with  subsequent  necrosis 
are  characteristic.  The  phalanges  gradually  fall  off.  The  process  may 
begin  at  the  nail  and  then  progressively  involve  the  entire  digit.  The 
skin  presents  patches  of  discoloration  of  bronze  color.  Small  areas  of 
thickened  skin  on  the  ears  and  face  have  been  observed  (due  to  a  local 
accumulation  of  bacilli). 

In  the  majority  of  cases  the  hands  present  a  claw-like  appearance 
with  marked  atrophy  of  Aran-Duchenne's  type  (see  this  chapter).     Reac- 


394 


MULTIPLE    NEURITIS 


tions  of  degeneration  are  present.     The  atrophy  may  not  be  confined  to  the 
extremities  but  also  involve  the  muscles  of  the  trunk  and  face.     Objective 

sensory  losses  are  usually  found  over 
the  distal  portions  of  the  limbs.  Syrin- 
gomyelic sensory  dissociation  is  common, 
but  at  the  beginning  there  is  a  long  period 
of  hyperesthesia  with  pain  in  the  ex- 
tremities. The  relationship  between 
syringomyelia  and  leprosy  is  according  to 
competent  authorities  a  very  intimate  one. 
Prognosis  is,  generally  speaking,  un- 
favorable, although  the  disease  may  last 
several  years.  The  disease  may  be  also 
self-limited. 

[  The  treatment,  is  mostly  hygienic. 
Alteratives,  also  strychnia,  may  be  tried. 
J.  Senile  Neuritis  has  been  described 
particularly  by  Oppenheim.  He  observed 
a  slight  paralysis  of  the  distal  ends  of  the 
limbs,  which  develop  very  gradually. 
Pain  and  hyperesthesia  may  or  may  not 
be  present.  The  degenerative  state  of 
the  peripheral  nerves  is  probably  due  to  the 
deficient  blood  supply  caused  by  athero- 
matous arteries  characteristic  of  old  age. 


Fig.  128. — Leprosy.  Muti- 
lated toes,  ulcerations,  self-amputa- 
tions, constrictions  around  phalan- 
ges. Patches  of  discoloration  of 
skin  of  the  face  and  feet. 


Fig.  129. — Leprosy  on  Hands. 


Diagnosis  of  Multiple  Neuritis. — The  above  described  general  symp- 
toms of  polyneuritis  and  the  special  symptoms  of  each  individual  form 


MULTIPLE    NEURITIS  395 

are  sufficiently  characteristic  for  establishing  the  diagnosis.  There  are, 
however,  a  few  maladies  which  sometimes  resemble  closely  polyneuritis. 
They  are:  Acute  myelitis,  anterior  poliomyelitis,  tabes. 

When  the  paralysis  due  to  myelitis  is  spastic,  the  disease  cannot  be 
confounded  with  polyneuritis,  as  the  state  of  the  tonicity  of  the  muscles, 
the  reflexes,  the  gait  and  the  condition  of  the  sphincters  are  radically 
different  in  the  two  affections. 

The  flaccid  form  of  myelitis  presents  some  difficulty  of  differentia- 
tion. Here  the  anaesthesia  is  much  pronounced  and  affects  almost  sym- 
metrically the  entire  lower  extremities  (in  polyneuritis  the  objective 
sensory  disturbances  are  slight);  the  involvement  of  the  bladder  and 
rectum,  also  sexual  disturbances,  are  constant  (in  polyneuritis  they  are 
usually  absent);  bed-sores,  also  ulcerations  of  the  heels  are  constant 
(absent  in  polyneuritis) . 

In  acute  anterior  poliomyelitis  the  paralysis  affects  chiefly  the  roots 
of  the  limbs  while  the  distal  ends  are  not  involved  (in  polyneuritis  the 
condition  is  reversed);  the  cranial  nerves  are  rarely  affected  (in  poly- 
neuritis frequently) ;  fibrillary  contractions  are  present  (absent  in  poly- 
neuritis); sensory  disturbances  are  absent  (present  in  polyneuritis); 
the  distribution  of  paralysis,  of  atrophy  and  electrical  contractility  is 
symmetrical  (irregular  in  polyneuritis) . 

In  tabes  the  etiology  is  different  from  that  of  polyneuritis.  In  the 
former  the  presence  of  Argyll-Robertson's  pupils,  of  disturbances  of  the 
sphincters,  of  optic  atrophy,  gastric  crises,  exaggerated  superficial  ab- 
dominal reflexes,  thoracic  bands  of  anaesthesia,  finally  the  absence  of 
tenderness  of  the  nerve-trunks  (so  characteristic  of  polyneuritis)  will 
decide  the  diagnosis.  Lymphocytosis  of  the  cerebro-spinal  fluid  is  present 
in  tabes,  absent  in  polyneuritis. 

Treatment. — The  first  indication  is  to  remove  the  cause.  In  cases 
of  intoxication  with  alcohol  the  patient  should  be  isolated;  in  poisoning 
with  lead  or  mercury  the  patient  is  advised  to  discontinue  his  occupa- 
tion. In  diphtheritic  polyneuritis  administration  of  antitoxin  may  be 
of  benefit.  In  syphilitic  cases  mercury  and  iodides  are  indicated.  Iodides 
are  also  beneficial  in  lead  neuritis.  In  rheumatic  cases  salicylates, 
iodides,  diuretics,  diaphoretics  are  of  use.  When  pain  is  present,  it  will 
be  relieved  by  rest  in  bed,  hot  applications  or  hot  baths,  also  by  coal-tar 
products  (aspirin,  phenacetin,  etc.),  sedatives  (bromides).  Morphia 
should  be  avoided.  When  the  acute  stage  has  subsided,  massage  and 
electricity  will  be  very  useful  for  combating  the  oncoming  atrophy  and 
perhaps  for  relieving  the  pain.  As  soon  as  pain  has  subsided,  the 
patient  should  be  allowed  to  use  his  limbs  and  this  is  aided  by  daily 


39^  MULTIPLE   NEURITIS 

massage,  electricity  and  systematic  exercises  especially  in  cases  of  in- 
coordination. When  permanent  contractures  with  deformities  are 
present,    surgical  intervention  is  necessary. 

General  hygiene,  good  nutritious  food  are  essential,  and  in  case  of 
emaciation,  iron  and  tonics  should  be  added. 

ACUTE  ASCENDING  PARALYSIS  (LANDRY'S  PARALYSIS) 

Pathology. — The  findings  in  Landry's  paralysis  are  not  constant. 
Frequently  are  seen  changes  in  the  spinal  cord.  In  such  cases  there  may 
be  disseminated  inflammatory  foci  (myelitis),  or  a  certain  degree  of 
anterior  poliomyelitis  or  else  changes  in  the  pyramidal  tracts.  The  cells 
of  the  anterior  cornua  appear  smaller,  their  contour  is  irregular.  Their 
chromatolysis  is  marked.  The  most  pronounced  changes  in  the  majority 
of  cases  are  seen  in  the  lumbar  enlargement,  which  corresponds  clinically 
to  the  earliest  involvement  of  the  lower  limbs.  The  axis  cylinders  and 
their  myelin  sheaths  are  in  a  state  of  degeneration.  The  neuroglia 
cells  are  smaller  and  in  a  state  of  degeneration;  sometimes  hyperplasia 
is  present.  The  blood  vessels  may  be  normal  or  more  frequently  present 
a  leucocytic  infiltration;  in  the  latter  case  rupture  may  take  place;  puncti- 
form  hemorrhages  are  not  rare.  The  roots,  especially  the  anterior  ones,, 
and  the  peripheral  nerves  are  involved.  Disintegration  of  myeline  and 
subsequently  atrophy  of  the  axis  cylinders  are  characteristic.  When 
the  meninges  are  involved,  they  present  all  degrees  of  inflammation. 
The  alterations  just  described  are  not  all  present  in  every  case.  In  some 
cases  there  are  only  central  changes.  In  some  cases  there  are  evidences 
of  neuritis.  In  other  cases  there  may  be  a  combination  of  spinal  and 
neuritic  lesions  with  or  without  involvement  of  the  medulla.  The  ob- 
served alterations  are  variable  as  to  their  localization  and  intensity. 
Acute  ascending  paralysis  is  an  affection  of  the  peripheral  motor  neurone 
involving  separately  or  simultaneously  every  constituent  portion  of  the 
neurone.  Finally  in  a  certain  number  of  cases  no  lesion  whatever  has 
been  discovered. 

The  cerebro-spinal  fluid  presents  also  a  variability  in  findings.  The 
leucocytic  formula  may  be  either  normal  or  exaggerated.  Lymphocy- 
tosis or  polymorphonucleosis  have  been  found.  However  it  may  be 
said  with  some  degree  of  certainty  that  abundant  leucocytosis  is  in  favor 
of  central  origin  (spinal  cord)  of  the  disease,  while  absence  of  leucocytosis 
is  in  favor  of  its  neuritic  origin. 

Etiology. — All  the  evidences  point  to  an  infectious  origin  of  the 
malady.  It  may  develop  in  the  course  of  various  infectious  diseases  or 
independently.     By  means  of  injections  o'f  cultures  in  animals  typical 


MULTIPLE    NEURITIS  397 

Landry's  paralysis  had  been  reproduced  experimentally  (Thoinot  and 
Masselin).  The  microorganism  has  not  yet  been  discovered.  A  recent 
case  reported  by  Bolten  (Berl.  klin.  Wchn.,  Jan.,  191 1)  seems  to  confirm 
the  contention  that  Landry's  paralysis  is  the  result  of  intoxication  of 
the  spinal  cord,  the  toxin  interfering  with  the  function  of  the  spinal  and 
bulbar  centers.  The  toxin  seems  to  affect  the  motor  neurone  alone.  On 
this  basis  600  c.c.  of  cerebro-spinal  fluid  were  withdrawn  and  substituted 
by  540  c.c.  of  non-toxic  fluid,  viz.  salt  solution.     The  patient  recovered. 

Among  other  etiological  factors  may  be  mentioned:  cold  or  extreme 
heat.  Men  are  more  affected  than  women,  adults  more  frequently  than 
children  or  aged  individuals. 

Symptoms.- — The  clinical  picture  may  be  either  that  of  poliomyelitis 
or  polyneuritis.  There  is  almost  always  a  prodromal  stage  lasting  from 
hours  to  weeks.  General  malaise,  pain  in  the  back  and  various  parses- 
thesias  (tingling,  numbness)  in  the  limbs  usually  precede.  The  onset  is 
always  rapid  or  even  sudden.  The  disease  in  typical  cases  begins  with 
paralysis  of  the  lower  extremities.  Rapidly  the  upper  extremities  and 
the  trunk  become  invaded.  They  are  followed  by  the  muscles  of  the 
head,  neck,  the  tongue,  palate,  larynx  and  pharynx.  Difficulty  of  deg- 
lutition and  of  respiration,  slowness  or  else  acceleration  of  the  pulse 
appear.  The  patient  dies  from  asphyxia.  Ocular  palsies,  as  well  as 
facial  palsy,  have  been  observed  in  some  cases. 

The  paralysis  is  flaccid.  The  reflexes,  deep  and  superficial,  are 
abolished.  Sensations  are  very  slightly  or  not  at  all  disturbed.  The 
sphincters  are  usually  intact.  The  muscles  are  usually  normal  and  no 
reactions  of  degeneration  are  present.     Fever  is,  as  a  rule,  absent. 

In  atypical  cases  the  onset  of  paralysis  may  be  preceded  by  chills, 
headache,  malaise.  The  paralysis  of  the  lower  extremities  is  gradually 
followed  by  involvement  of  the  sphincters  and  of  the  upper  extremities 
and  sometimes  of  the  medulla.  Among  the  atypical  cases  can  also  be 
mentioned  those  in  which  the  paralysis  begins  with  the  upper  extremities. 

In  another  series  of  cases  there  may  be  also  the  typical  picture  of  multi- 
ple neuritis  with  pain  and  tenderness  of  the  nerve-trunks,  etc. 

Course,  Termination,  Prognosis. — In  the  majority  of  cases  the 
termination  is  fatal.  Recovery  rarely  occurs.  In  the  latter  case  the 
symptoms  gradually  improve.  The  patient  may  recover,  particularly 
when  the  bulbar  symptoms  disappear.  In  such  cases  muscular  atrophy 
will  be  observed;  it  makes  its  appearance  at  the  end  of  a  few  weeks. 
The  atrophy  is  then  diffuse.  Reactions  of  degeneration  are  absent;  there 
is  only  a  diminution  of  response  to  electric  stimulation.  In  fatal  cases 
the  duration  is  only  of  a  few  days  (three  to  eight) . 


39§  MULTIPLE    NEURITIS 

Diagnosis. — The  rapidity  of  invasion,  the  mode  of  development  of  the 
paralysis,  viz.  the  ascending  course,  are  characteristic  of  the  disease. 
From  acute  anterior  poliomyelitis  it  will  be  differentiated  by  the  rapid 
course  and  bulbar  involvement.  Besides,  the  prodromal  symptoms  such 
as  malaise,  gastro-intestinal  disturbances,  etc.,  are  more  pronounced  in 
acute  poliomyelitis.  Asymmetry  in  the  involvement  of  muscles  on.  both 
sides  of  the  body  is  characteristic  of  poliomyelitis.  Rapid  improvement 
in  some  muscles  within  a  few  days,  early  atrophy  and  RD  are  typical 
only  of  poliomyelitis. 

Treatment. — At  the  appearance  of  the  first  symptoms  the  chief  in- 
dication is  elimination  of  the  infectious  element.  This  can  be  accom- 
plished to  some  extent  by  proper  elimination.  Diuretics,  purgatives  and 
diaphoretics  should  be  administered.  Cauterization  of  the  spinal  column 
is  advisable.  Internally  ergot,  and  in  specific  cases,  mercury  may  be  of 
some  use.  If  the  patient  survives,  and  paralysis  with  atrophy  becomes 
chronic,  massage  and  electricity  are  useful. 

PERIODIC  PARALYSIS 

Westphal  in  1885  first  called  attention  to  an  affection  which  is  analo- 
gous to  polyneuritis.  It  is  characterized  by  attacks  of  paralysis  in  the 
intervals  of  which  the  patient  is  perfectly  normal.  The  attacks  may  last 
from  several  hours  to  two  days.  The  palsy  affects  all  voluntary  muscles 
except  those  of  the  face,  tongue,  pharynx,  mouth,  eyes,  also  those  of  the 
sphincters.  The  lower  as  well  as  the  upper  extremities  may  be  equally 
involved,  although  the  latter  more  frequently  than  the  first.  The  paraly- 
sis develops  rapidly  and  it  is  flaccid.  It  is  usually  preceded  by  a  tingling 
sensation  and  even  some  pain.  As  a  rule  the  lower  extremities  are  first 
attacked,  then  follow  the  trunk  and  arms.  Sometimes  some  muscles  of 
the  neck  are  affected:  the  patient  can  move  his  head  when  it  lies  on  a  pil- 
low, but  he  cannot  raise  it.  The  muscles  innervated  by  the  cranial 
nerves  are  intact.  The  sphincters  are  intact  and  if  constipation  is  pres- 
ent, it  is  due  to  a  paralysis  of  the  abdominal  muscles.  The  return  of 
power  is  done  in  a  manner  reverse  to  the  development  of  paralysis.  Thus 
the  arm  first  and  the  legs  last  regain  their  motility.  The  reflexes  are 
abolished  or  markedly  diminished.  The  electrical  contractility  of  mus- 
cles and  nerves  is  markedly  diminished  or  totally  abolished,  although  the 
muscles  may  contract  when  the  nerves  are  being  stimulated.  Sensations 
and  pupillary  reflexes  are  normal.  A  very  frequent  symptom  is  sweat- 
ing during  the  attacks  which  may  last  between  fifteen  minutes  and  two  days. 

The  attacks  may  be  severe  or  complete  and  mild  or  incomplete.  In 
the  latter  case  there  is  only  paresis  of  the  muscles. 


DISEASES    OF    CRANIAL   NERVES  399 

As  to  less  frequent  symptoms,  there  may  be  disturbance  of  circulation: 
dilatation  of  the  heart;  weak  and  irregular  pulse;  pallor  of  the  face;  ex- 
cessive thirst;  congestion  of  the  conjunctivae  and  of  the  respiratory  tract, 
slight  spasm  of  the  affected  muscles.     Consciousness  is  preserved. 

The  etiology  is  not  definite.  The  hereditary  character  of  the  disease 
is  well  known.  Holtzapple  reports  seventeen  cases  in  four  generations. 
The  exciting  causes  may  be  either  rest  or  exertion.  In  the  first  case  the 
attacks  occur  after  a  night's  sleep  or  after  the  patient  is  seated  and  rests  a 
certain  time.  In  the  second  case  violent  exertion  brings  on  an  attack.  In 
one  of  my  patients,  girl  of  fourteen,  an  attack  occurred  after  a  long  walk 
or  dance;  she  would  develop  paralysis  first  in  the  arms  and  then  in  the 
legs;  it  lasted  from  twenty-five  minutes  to  eight  hours.  Her  uncle  was 
similarly  affected.     Holtzapple  observed  attacks  after  indulgence  in  food. 

The  prognosis  as  a  rule  is  favorable  to  life,  because  the  attacks  become 
less  frequent  with  advancing  age.  Holtzapple,  however,  observed  six 
deaths  during  attacks.  Difficult  respiration  during  an  attack  is  of  serious 
moment. 

The  pathogenesis  of  the  disease  is  unknown.  A  diminished  secre- 
tion of  urea  was  found  in  several  members  of  the  same  family.  Necrop- 
sies have  been  negative.  The  trouble  probably  lies  in  a  developmental 
defect  of  the  muscles  themselves. 

Treatment.— Avoidance  of  overexertion,  of  stimulants,  of  over-feeding 
is  indicated.  In  my  case  mentioned  above  I  succeeded  in  shortening 
the  attacks  with,  the  administration  of  sodium  bromide  in  gr.  x  doses 
every  two  hours.  Massage  was  also  used  in  my  case.  The  results  were 
satisfactory. 

DISEASES  OF  INDIVIDUAL  NERVES 

I.  Paralysis  of  Cranial  Nerves 

A.  Paralysis  of  the  First  Nerve  (Olfactory).  Symptoms. — Loss  or 
impairment  of  the  sense  of  smell  (anosmia)  is  the  prominent  symptom. 
As  the  taste  is  dependable  to  a  large  extent  upon  the  integrity  of  smell, 
the  former  will  be  involved  when  anosmia  is  present.  When  the  olfactory 
nerve  is  in  a  state  of  irritation,  hallucinations  of  smell  (parosmia)  may 
occur.  Subjective  sensations  of  smell  may  occur  as  an  aura  preceding 
epileptic  seizures  in  cases  of  tumor  of  the  uncinate  portion  of  temporo- 
sphenoidal  lobe. 

Etiology. — Diseases  of  the  nasal  cavities  (in  the  upper  portion  of  which 
are  distributed  the  fine  olfactory  nerves),  viz.  tumors,  catarrh,  dis- 
eases of  the  bony  structure,  meningitis  at  the  base,  trauma  of  the  nose, 


400  DISEASES    OE    CRANIAL   NERVES 

fracture  at  the  base  of  the  skull  in  the  anterior  fossa — are  all  apt  to  irritate 
or  destroy  the  filaments  of  the  olfactory  nerve. 

Treatment. — Removal  of  the  cause  is  the  sole  therapeutic  indication. 

B.  Diseases  of  the  Second  Nerve  (Optic)  are  discussed  in  the  chapter 
on  Diseases  of  the  Brain. 

Optic  Neuritis  and  optic  atrophy  were  mentioned  and  their  significance 
emphasized  in  intra-cranial  diseases  (especially  tumors,  abscess  of  the 
brain,  basilar  meningitis,  choked  disc  in  cerebellar  tumors),  in  tabes, 
insular  sclerosis,  Freidreich's  ataxia,  paresis.  It  may  be  added  here  that 
optic  neuritis  may  be  encountered  also  in  acute  febrile  diseases,  toxic 
conditions  (see  polyneuritis),  anaemia,  diseases  of  orbital  region,  Bright's 
disease,  gout.  Optic  atrophy  may  also  develop  in  syphilis,  malaria, 
diabetes,  abuse  of  tobacco,  narcotics  or  other  drugs  (quinine). 

In  optic  neuritis  the  disc  is  swollen  and  hyperemic.  When  the  veins 
become  greatly  distended  and  oedema  develops  because  of  the  interference 
with  the  return  circulation,  the  condition  is  "choked  disc."  Vision  may 
remain  intact  in  optic  neuritis  and  even  in  choked  disc.  On  the  other 
hand,  there  may  be  various  degrees  of  impairment  of  vision  or  complete 
loss  of  vision. 

Loss  of  vision  (amaurosis)  may  occur  in  the  course  of  infectious  diseases 
and  develop  very  rapidly.  Widal,  Joltrain  and  Weill  (Presse.  Med.,  1909) 
report  a  case  of  amaurosis  in  typhoid  fever  accompanied  by  cedema  of 
both  papillae  and  hypertension  of  the  cerebro-spinal  fluid.  Two  lumbar 
punctures  produced  complete  recovery  from  the  eye  symptoms. 

Optic  atrophy  may  be  primary  like  in  tabes  or  multiple  sclerosis,  or 
secondary  following  optic  neuritis.  In  primary  optic  atrophy  the 
loss  of  vision  is  usually  gradual.  Besides,  there  may  be  loss  of  vision 
only  in  one  portion  of  the  visual  field,  either  centrally  or  peripherally. 

Neuritis  and  atrophy  of  the  optic  nerve  may  be  the  result  not  only  of 
organic  diseases  of  the  nervous  system  (see  above),  but  also  of  localized 
disturbances  in  the  nerve  itself,  such  as  embolism  of  the  central  artery, 
hemorrhage  and  trauma. 

The  pupillary  reactions  are  frequently  affected  in  diseases  of  the 
optic  nerve;  impairment  or  loss  of  light  reflexes  is  common. 

Diseases  of  the  optic  chiasm  were  discussed  in  the  chapter  on  Hemi- 
anopsia. 

C.  Paralysis  of  Third,  Fourth  and  Sixth  Nerves  (Opthalmoplegia.) 
Symptoms. — They  are  general  and  special  to  each  of  the  three  nerves. 

General. — Strabismus  is  very  conspicuous.  It  consists  of  a  deviation 
of  the  eye  globe.  In  the  view  of  the  paralysis  the  excursion  of  the  latter  is 
limited.     For  example,  if  the  external  rectus  (sixth  nerve)  is  involved,  the 


DISEASES    OF    CRANIAL   NERVES  40 1 

eye  globe  will  move  in  every  direction  except  externally.  Under  secondary 
deviation  is  understood  a  deviation  of  the  normal  eye  when  the  paralyzed 
eye  endeavors  to  fix  an  object.  This  secondary  deviation  is  always  greater 
than  the  paralytic  deviation. 

The  consequence  of  strabismus  is  double  vision  (diplopia)  because 
the  image  of  an  object  will  be  formed  in  both  retime  at  various  levels. 
Diplopia  produces  not  infrequently  vertigo  and  headache.  Vertigo  may 
disappear  when  the  vision  of  the  diseased  eye  is  entirely  removed  or  else 
corrected  by  some  means. 

Special  Symptoms,  (a)  Paralysis  of  the  Third  Nerve  (Oculomotor.) — 
It  may  be  complete  or  partial. 

In  complete  palsy  the  levator  palpebral  superioris,  the  superior, 
inferior  and  internal  recti,  the  inferior  oblique,  the  sphincter  of  the 
pupil  and  the  ciliary  muscles  are  all  involved.  Ptosis  is  the  striking 
symptom.  The  upper  eye-lid  is  lowered.  The  eye  globe  is  deviated 
externally  and  downward.  The  pupil  is  dilated,  does  not  react  to  light 
or  to  accommodation.  Diplopia  is  marked:  one  image  is  placed  higher 
than  the  other. 

In  partial  paralysis  any  one  of  the  muscles  may  be  affected.  When  the 
internal  rectus  alone  is  paralyzed,  there  is  divergent  strabismus:  the  images 
in  both  eyes  are  at  the  same  level.  When  the  inferior  rectus  is  paralyzed, 
the  eye  looks  upward  and  outward,  the  head  is  lowered.  In  paralysis  of 
the  superior  rectus,  the  head  is  raised  and  thrown  backward.  When  the 
inferior  oblique  is  involved,  the  eye  is  directed  downward  and  inward. 

In  nuclear  lesions  of  the  third  nerve  the  internal  muscles  may  escape, 
but  the  external  are  paralyzed. 

(b)  Paralysis  of  the  Fourth  Nerve  (Pathetic).— The  superior  oblique 
is  involved.  The  eye  globe  looks  upward  and  inward.  Diplopia  exists 
only  in  the  lower  part  of  the  visual  field. 

(c)  Paralysis  of  the  Sixth  Nerve  (Abducens).- — The  external  rectus  is 
involved.     The  eye  globe  is  turned  inward  (convergent  strabismus). 

Ocular  palsies  may  be  confined  to  one  nerve  or  to  all  the  three  nerves. 
As  to  the  third  nerve  several  branches  may  be  affected  at  the  same 
time.  Ophthalmoplegia  is  present  when  all  the  muscles  of  the  eye  are 
involved  or  at  least  the  muscles  innervated  by  two  nerves,  one  of  which 
is  the  third  nerve.  In  external  ophthalmoplegia  the  extrinsic  muscles  of 
the  eye,  in  internal  ophthalmoplegia  the  intrinsic  muscles  (the  sphincters 
of  the  pupil  and  the  ciliary  muscles)  are  paralyzed. 

Ophthalmoplegia  may  be  nuclear  and  peripheral.  The  first  is  the 
most  important.  When  it  is  nuclear  and  external,  it  is  usually  bilateral. 
The  facies  of  the  patient  is  then  characteristic:  the  ptosis  gives  a  drowsy, 
26 


402  DISEASES    OF   CRANIAL   NERVES 

sleepy  expression,  the  eye  globes  are  immobile,  but  the  pupillary  and 
accommodation  reflexes  are  normal.  Alternating  ptosis  is  quite  frequent. 
When  the  ophthalmoplegia  is  nuclear  and  internal,  mydriasis  is  present 
and  the  pupils  do  not  react  to  light,  to  accommodation  and  to  convergence. 

Pupillary  reactions.  Iridoplegia  is  present  when  the  light  reflex  of  the 
pupil  is  lost.  If  with  the  latter  the  accommodation  reflex  is  preserved, 
we  are  in  presence  of  Argyll-Robertson  pupil.  The  lesion  in  this  phe- 
nomenon is  very  probably  in  the  ciliary  ganglion  according  to  the 
researches  of  Marina  and  others. 

Paralysis  of  accommodation  or  Cycloplegia  is  met  with  in  syphilis 
and  diphtheria;  it  is  due  to  a  palsy  of  the  third  nerve. 

Associative  Paralysis:  (i)  Paralysis  of  Conjugate  Movement. — It 
consists  of  abolition  of  a  movement  common  to  both  eyes,  for  example, 
paralysis  of  internal  rectus  of  one  eye  and  of  external  rectus  of  the  other. 
This  form  is  not  produced  by  a  paralysis  of  the  nerves  themselves,  but  by 
a  lesion  of  the  sixth  nucleus  which  besides  his  numerous  other  connections 
it  is  associated  through  the  posterior  longitudinal  bundle  with  that  part 
of  the  third  nucleus  which  supplies  the  internal  rectus  muscle  of  the  op- 
posite eye.  (2)  Paralysis  of  convergence  of  internal  recti  of  both  eyes  is 
probably  due  to  a  lesion  of  the  nuclei  of  the  third  nerve.  It  is  usually 
associated  with  paralysis  of  accommodation. 

Ocular  palsies  are  frequent  in  tabes.  Their  onset  is  sudden  and 
characterized  frequently  by  a  paralysis  of  the  third  nerve  and  their 
predilection  for  the  pupil  (Argyll-Robertson). 

Course,  Termination,  Prognosis. — They  depend  upon  the  cause.  The 
course  is  variable,  it  may  disappear  and  recur.  In  an  advanced  stage  of 
tabes  it  may  remain  permanently.  In  syphilis  ocular  palsies  are  slow  in 
onset,  but  progressive.  They  also  affect  chiefly  the  third  nerve.  Like 
in  tabes  they  are  peripheral  in  origin.  They  may  be  promptly  ameliorated 
by  salvarsan.  mercury  and  iodides.  In  multiple  neuritis  ocular  palsies 
disappear  when  the  other  symptoms  improve.  Ocular  palsies  following 
infectious  diseases  usually  recover.  In  tumors,  hemorrhages,  fractures, 
the  ocular  palsies  are  incurable.    Nuclear  palsies  do  not  recover. 

Etiology. — Diseases  of  the  brain  may  produce  a  conjugate  deviation 
of  the  eyes,  also  a  paralysis  of  the  levator  palpebral  (blepharoptosis). 
Tumors  or  other  lesions  at  the  base  of  the  brain  frequently  produce  ocular 
palsies  in  addition  to  crossed  hemiplegia.  In  Polioencephalitis  superior 
(see  this  chapter)  nuclear  ophthalmoplegia  is  a  frequent  accompaniment. 
In  tabes  and  in  paresis  the  third  nerve  is  frequently  involved.  The  same 
is  observed  occasionally  in  multiple  sclerosis  and  in  syringomyelia.  In 
polyneuritis    (see   this   chapter)    ophthalmoplegia  is   observed.     Various 


DISEASES    OF   CRANIAL   NERVES  403 

infectious  diseases,  intoxications  may  be  the  cause  of  palsy  of  the  ocular 
muscles.  Syphilis  plays  a  prominent  role  in  the  causation  of  this  paralysis. 
Traumatism  of  the  orbital  region,  compression,  fractures  at  the  base  of  the 
brain,  aneurisms  of  the  arteries  at  the  base,  thrombosis  of  the  cavernous 
sinus,  tubercular  meningitis  or  gummata  at  the  base  will  all  involve  the 
motor  ocular  nerves.  Sinusitis,  frontal  and  ethmoidal,  may  be  the  cause 
of  ocular  palsies  either  directly  or  through  an  inflammatory  condition  of 
the  orbit.     Finally  ocular  palsies  may  be  congenital. 

Treatment. — Removal  of  the  cause  is  the  first  indication.  In  syphilis 
mercury  and  iodides  are  necessary.  In  toxic  or  infectious  cases  blood- 
letting and  sweating  may  be  of  benefit.  Hypodermic  injections  of  strych- 
nia yield  sometimes  good  results.  Electrical  stimulation  of  the  palsied 
muscles  may  be  useful.  Diplopia  can  be  corrected  either  by  special 
glasses  or  surgical  means.     The  tendency  to  remissions  is  great. 

Nystagmus.— It  is  an  associated  oscillation  of  the  eyes  which  is 
accomplished  symmetrically  and  more  or  less  rapidly.  Most  frequently 
the  nystagmus  is  horizontal,  sometimes  it  is  vertical.  It  may  be  also 
rotatory.  In  the  majority  of  cases  nystagmus  is  a  convulsive  phenomenon 
occurring  mostly  upon  voluntary  movement  of  the  eyes.  It  is  due  to  an 
irritative  lesion  of  the  center  which  controls  associative  movements.  In 
exceptional  cases  it  is  unilateral. 

Nystagmus  may  be  congenital  or  hereditary.  It  may  be  present 
without  any  eye  lesion  or  else  it  may  accompany  cataract,  albinism, 
coloboma,  strabismus,  errors  of  refraction. 

Myoclonic  nystagmus  described  by  Lenoble  and  Aubineau  is  a  con- 
genital condition  consisting  of  nystagmus  accompanied  by  facial  asym- 
metry and  tremor  of  the  muscles  of  the  face  and  increased  reflexes. 

Nystagmus  is  most  frequently  acquired.  It  may  be  the  result  of 
impaired  vision  and  when  the  latter  improves,  the  former  disappears. 
Occasionally  it  is  observed  in  normal  individuals  during  fixation  of  the 
eyes  on  an  object.  It  is  met  with  in  the  following  diseases  of  the  central 
nervous  system  :  Multiple  sclerosis,  Friedreich's  disease,  heredo-cerebellar 
ataxia,  syringomyelia,  very  frequently  in  cerebellar  diseases,  in  lesions  of 
the  quadrigeminal  bodies.  In  diseases  of  the  medulla  when  the  lesion 
extends  to  the  pons  nystagmus  is  observed.  Here  the  posterior  longi- 
tudinal bundle  or  the  centers  of  association  are  involved.  Thus  it  is 
met  with  in  Pseudo-bulbar  palsies  and  in  tumors  or  focal  lesions  of  the 
pons.  In  diseases  of  the  brain  nystagmus  may  occur.  It  may  depend 
upon  the  distant  effect  of  the  cerebral  lesion  on  the  posterior  cerebral 
fossa.  It  may  depend  also  on  a  lesion  of  the  cortical  areas  which  control 
the  movements  of  the  eyes,  viz:  frontal  lobe,  angular  gyrus,  visual  area. 


404  DISEASES    OF   CRANIAL   NERVES 

Vestibular  Nystagmus.- — The  involvement  of  the  labyrinth  causes 
nystagmus.  The  reflex  is  transmitted  to  the  oculo-motor  nerve  from  the 
vestibular  apparatus  through  the  posterior  longitudinal  bundle  and 
cerebellar  peduncles.  The  nystagmus  is  due  to  a  displacement  of  the 
endo-lymph  fluid  in  the  semicircular  canal.  Horizontal  nystagmus  is  caused 
by  excitation  of  the  horizontal  canal,  vertical  by  excitation  of  the  superior 
vertical  canal,  rotatory  by  excitation  of  the  posterior  vertical  canal.  Barany  's 
test  consists  of  rotation,  caloric  and  galvanic  excitation.  Inrotation  (patient 
is  seated  on  a  turning  chair,  head  erect)  the  nystagmus  is  in  a  direction  of 
the  rotatory  movement,  but  as  soon  as  the  rotation  is  interrupted,  the 
nystagmus  is  the  opposite  direction.  Nystagmus  of  thermic  origin  pro- 
duced by  irrigation  of  the  ear  is  in  a  direction  opposite  to  the  irritated  ear 
if  the  water  is  cold  and  vice  versa  if  warm  water  is  used.  In  galvanic  test 
if  the  cathode  is  applied  to  the  mastoid,  rotatory  nystagmus  is  in  the  direc- 
tion to  the  same  side.  If  the  anode  is  applied,  the  nystagmus  is  to  the 
opposite  side. 

When  the  labyrinth  is  destroyed,  the  above  tests,  especially  the  caloric 
test,  will  not  be  able  to  produce  the  least  nystagmoid  movement  of  the 
eye  globes.  Absence  of  reflex  therefore  is  an  indication  of  disease  of 
the  labyrinth. 

Barany  has  observed  that  60  per  cent,  of  normal  individuals  present 
a  slight  nystagmus  on  looking  to  the  extreme  right  or  left.  In  labyrinthine 
disease  on  one  side  the  nystagmus  is  very  evident  when  the  eyes  are 
turned  to  one  or  the  other  side,  while  normally  it  is  present  on  looking  to 
either  side.  Besides,  pathological  nystagmus  is  generally  well  developed, 
while  normally  it  is  very  imperfect.  Continuous  nystagmus  associated 
with  vertigo  is  present  in  purulent  disease  of  the  labyrinth.  In  cerebellar 
abscess  there  is  also  nystagmus  associated  with  vertigo,  but  here  the 
nystagmus  is  directed  to  the  side  of  the  lesion,  while  in  labyrinthine 
diseases  to  the  sound  side. 

Vestibular  nystagmus  differs  from  nystagmus  of  multiple  sclerosis  or 
of  other  ocular  disorders  in  that  the  former  consists  of  unequal  oscilla- 
tions, one  movement  being  less  rapid  than  the  other  and  the  oscillations 
become  increased  when  the  eyes  are  turned  toward  the  shorter  movement, 
diminished  if  the  eyes  are  turned  toward  the  slower  movement. 

Miners'  nystagmus  is  an  occupation  disorder  occurring  in  individuals 
working  in  coal  mines.  It  is  of  the  rotatory  form.  There  is  a  benign  variety 
which  does  not  interfere  with  work  and  a  grave  variety  which  is  much  less 
frequent.  The  nystagmus  occurs  in  the  benign  cases  only  when  the  eyes 
are  directed  upward.  In  the  severe  cases  it  will  occur  when  the  eyes  are 
directed  downward.     The  individual  sees  objects  moving  continuously 


DISEASES    OF   CRANIAL   NERVES  405 

before  him  (dancing)  which  of  course  prevents  him  from  working.  The 
cause  if  this  nystagmus  lies  in  forced  elevation  and  oblique  direction  of 
the  eyes  and  poor  light  in  the  mines. 

Perhaps  the  displacement  of  labyrinthine  endo-lymph  caused  by  forced 
elevation  of  the  head  is  the  cause  of  the  nystagmus. 

Hippus.— It  consists  of  alternating  dilatation  and  contraction  of  the 
pupils.  It  sometimes  accompanies  nystagmus.  It  is  probably  due  to  a 
dynamic  disturbance  of  equilibrium  in  the  movements  of  the  iris. 

D.  Paralysis  of  the  Fifth  Nerve  (Trigeminal).  Symptoms. — As  the 
fifth  nerve  consists  of  sensory  and  motor  portions  the  symptoms  will  be 
sensory  and  motor. 

Sensory.— The  area  of  the  skin  supplied  by  the  nerve  will  be  anaesthetic, 
viz.  conjunctiva,  cornea,  cheek,  nose,  lips,  mouth,  gums  and  tongue. 
The  external  auditory  meatus  and  the  skin  over  the  largest  portion  of  the 
lower  maxilla  are  not  involved.  The  corneal  and  lid  reflexes  are  abolished. 
The  secretions  of  the  eyes  and  nose  are  impaired.  Smell  and  taste  are 
also  impaired.  Trophic  disturbances  are  frequent.  Herpes  is  common 
and  herpes  zoster  ophthalmicus  is  due  to  neuritis  of  the  first  branch  of  the 
fifth  nerve. 

Neuroparalytic  Keratitis  is  not  infrequent.  It  is  characterized  by  an 
inflammation  and  ulceration  of  the  eye.  According  to  the  latest  view  on 
this  affection  the  keratitis  is  not  due  to  a  disease  of  supposed  trophic 
fibers  of  the  fifth  nerve,  but  to  an  irritation  of  the  trigeminal  fibers. 

Motor. — The  masticatoy  muscles  (masseter,  temporal  and  pterygoids) 
are  paralyzed  and  in  unilateral  paralysis  the  tongue  instinctively  pushes 
the  food  toward  the  normal  side  in  the  act  of  mastication.  The  unilateral 
involvement  of  the  pterygoid  muscles  produces  a  deviation  of  the  lower 
jaw  to  the  paralyzed  side  when  the  mouth  is  opened.  The  affected  muscles 
undergo  atrophy  and  in  this  case  they  show  reactions  of  degeneration. 

When  the  paralysis  of  the  fifth  nerve  is  incomplete,  as  it  happens  in 
cases  of  compression,  the  sensory  and  motor  symptoms  are  also  incomplete. 
There  will  be  only  diminished  sensations  (hypaesthesia)  instead  of  anaes- 
thesia, but  the  continuous  irritation  from  the  compression  will  produce 
pain.  If  the  condition  continues,  complete  anaesthesia  will  finally  develop. 
In  diseases  of  the  pons  the  spinal  rcot  of  the  fifth  nerve  may  be  involved; 
there  will  be  facial  anaesthesia  on  the  same  side  and  paralysis  of  the  arm 
and  leg  on  the  opposite  side. 

The  prognosis  depends  upon  the  original  cause. 

Etiology. — The  fifth  nerve  is  rarely  affected  primarily.  It  usually 
becomes  involved  in  the  course  of  other  diseases.  Lesions  at  the  base  of 
the  brain  (meningitis,  syphilis,  tumors,  hemorrhages,  aneurism),  otitis 


406  DISEASES    OF   CRANIAL   NERVES 

media,  caries  of  the  sphenoid,  trauma  of  the  orbits  or  of  the  maxillae — are 
all  causes  of  disease  of  the  fifth  nerve.  It  is  rarely  involved  in  polyneuritis. 
It  may  also  be  affected  in  tabes,  in  syringomyelia  and  its  motor 
nucleus  in  bulbar  palsy.  Unilateral  trigeminal  palsy  associated  with 
paralysis  of  the  eighth  nerve  is  frequently  met  with  in  tumor  of  the  ponto- 
cerebellar angle  (see  this  chapter). 

Treatment. — Removal  of  the  cause  is  the  first  indication.  Specific 
drugs  should  be  administered  when  syphilis  is  suspected.  Operations 
for  removal  of  growths,  etc.,  will  be  resorted  to  when  medications  fail. 
Pain  and  atrophic  disturbances  will  be  treated  with  appropriate  remedies. 
The  eye  should  be  protected  against  injury  or  irritation. 

E.  Paralysis  of  the  Seventh  Nerve  (Facial)  (Bell's  Palsy). — Facial 
palsy  may  be  of  cerebral,  nuclear  and  peripheral  origin.  The  latter  two 
are  identical  in  their  manifestations.  The  description  that  follows  will 
be  that  of  peripheral  facial  palsy.  In  discussion  on  diagnosis  the  differ- 
ential symptoms  of  cerebral  facial  palsy  will  be  given. 

Etiology. — Cold  is  a  frequent  cause  (rheumatic  paralysis).  Trauma 
is  next  in  frequency.  It  may  be  produced  by  fracture  of  the  petrous 
bone,  by  a  blow  or  operative  procedures  over  the  parotid  region,  and  in 
obstetrical  cases  by  pressure  of  the  nerve  against  the  pelvis  or  else  by  the 
forceps.  Lesions  in  the  vicinity  of  the  nerve,  as  tumor,  abscess,  exostosis, 
caries  of  the  petrous  bone,  otitis  media  are  apt  to  affect  the  nerve.  Infec- 
tious diseases  and  intoxications  are  occasionally  accompanied  by  facial 
palsy.  In  the  tertiary  period  of  syphilis  it  may  be  encountered.  An 
isolated  involvement  of  the  seventh  nerve  in  the  course  of  syphilis  is 
rare.  In  the  Archives  of  Diagnosis,  1908, 1  published  records  of  six  cases 
in  which  Bell's  palsy  was  caused  by  syphilis.  In  two  patients  the  facial 
paralysis  occurred  as  early  as  two  months  after  the  initial  chancre.  It 
may  appear  in  the  course  of  polyneuritis,  alcoholic  or  lead.  It  may 
develop  in  syphilitic  meningitis  at  the  base  of  the  brain,  also  in  the  course 
of  tumors  of  the  cerebello-pontile  angle.  Neuropathic  predisposition  plays 
a  certain  role. 

Pathology. — The  morbid  changes  consist  of  a  parenchymatous  neuritis. 
The  usual  seat  of  the  lesion  is  either  in  the  Fallopian  canal  or  in  the  stylo- 
mastoid foramen,  or  else  at  its  exit  from  the  latter. 

Symptoms. — The  onset  of  facial  palsy  is  sudden  in  cases  due  to  cold. 
It  is  usually  preceded  by  pain  in  the  neck  or  back  of  the  ear.  It  develops 
slowly  when  caused  by  other  factors. 

In  Bell's  palsy  the  striking  symptom  is  asymmetry  of  the  face.  The 
latter  is  deviated  to  the  normal  side.  The  muscles  on  the  entire  paralyzed 
side  have  lost  their  normal  tonicity  and  the  voluntary  motility:  they  are 


DISEASES    OF   CRANIAL   NERVES 


407 


relaxed.  The  naso-labial  fold  is  only  slightly  marked  and  the  angle  of 
the  mouth  is  lowered.  The  wrinkles  disappear.  The  asymmetry  becomes 
more  evident  when  the  patient  attempts  to  laugh,  to  show  his  teeth. 
Blowing  and  whistling  are  impossible.  In  the  latter  acts  the  air  raises 
the  cheek.  Liquid  food  runs  out  of  the  mouth  on  the  paralyzed  side. 
The  forehead  on  the  affected  side  is  also  smooth  and  when  the  patient  is 
told  to  wrinkle  it,  the  muscles  on  the  paralyzed  side  remain  immobile 
(paralysis  of  the  frontal  muscle). 

The  eye  remains  widely  open  because  of  the  paralysis  of  the  orbicularis 
palpebrarum.  As  closure  of  the  eye  is  impossible,  the  conjunctiva  and 
cornea  being  continuously  exposed,  become  inflamed.  Bell  called  atten- 
tion to  the  rotation  of  the  eye  globe 
upward  and  outward  at  the  attempt  to 
close  the  eye. 

The  tongue  when  protruded  appears 
to  be  deviated.  The  taste  on  its  anterior 
two- thirds  is  sometimes  diminished  or 
abolished  (involvement  of  the  chorda 
tympani).  The  palate  is  relaxed  and 
the  uvula  deviated.  The  sense  of  taste 
is  perceived  by  the  mucous  membranes 
of  the  tongue  and  soft  palate  including 
the  palatine  arches.  The  anterior  two- 
thirds  of  the  tongue  are  supplied  by  the 
chorda  tympani,  the  posterior  third  by 
the  ninth  nerve,  the  soft  palate  by  a 
branch  fiom  Meckel's  ganglion.  The 
facial  nerve  may  be  regarded  as  a 
mixed  nerve.  It  has  a  ganglion  (gen- 
iculate).    The  sensory  fibers  are:   the 

great  superficial  petrosal,  the  small  superficial  petrosal  and  the  external 
petrosal  supplying  the  carotid  plexus  of  the  sympathetic.  The  geniculate 
ganglion  sends  fibers  running  with  the  seventh  nerve  in  the  Fallopian 
canal,  also  fibers  anastomosing  with  the  fifth  and  eighth  nerves.  The  sen- 
sory fibers  of  the  seventh  nerve  are  represented  in  the  auditory  nerve,  the 
tympanic  plexus  (supplied  by  the  petrosal  nerves),  the  auricle  and  the 
external  auditory  canal. 

R.  Hunt  (/.  Nerv.  and  Ment.  Dis.,  1907)  has  brought  out  a  syndrome 
of  the  sensory  branches  of  the  seventh  nerve  occuriing  in  facial  palsy. 
The  symptoms  are:  (1)  pain  in  the  ear  and  mastoid  region.  The  pain  may 
be  of  neuralgic  character.     Anaesthesia  or  hyperesthesia  in  the  region  of 


Fig.  130. 


4o8 


DISEASES    OF   CRANIAL   NERVES 


concha  and  external  auditory  meatus,  also  hypaesthesia  in  the  chorda 
tympani  distribution.  (2)  Herpetic  otalgia,  herpes  oticus.  The  eruption 
is  distributed  over  the  tympanum,  external  auditory  canal  and  concha. 
(This  is  the  herpetic  area  of  the  geniculate  ganglion.)  (3)  Reflex  facial 
twitchings  and  spasms. 

The  hearing  is  sometimes  affected.  There  may  be  also  hyperacuity 
of  hearing. 

The  electrical  contractility  of  the  paralyzed  muscles  is  very  important. 
While  in  some  cases  (mild)  it  is  normal,  in  others  it  is  altered  quantita- 
tively and  qualitatively.     Reactions  of  degeneration  are  present. 

Among  the  rare  symptoms  of  peripheral  facial  palsy  may  be  mentioned 
absence  or  diminution  of  sweating  of  the  skin  on  the  affected  side.     This 

is  observed  in  the  severe  forms. 
Vaso-motor  disturbances  are 
sometimes  observed. 

Course,  Termination,  Prog- 
nosis.— Facial  palsy  due  to  ob- 
stetrical causes  has  a  favorable 
course,  it  lasts  usually  from 
eight  to  fifteen  days.  When 
the  nerve  is  destroyed  by  a 
fractured  bone,  the  paralysis  is 
incurable.  In  cases  of  facial 
palsy  due  to  the  middle-ear 
diseases  the  outcome  is  serious : 
the  palsy  persists.  In  cases 
produced  by  cold  (the  most 
frequent  variety)  the  palsy 
may  be  mild  and  complete; 
recovery  follows  in  from  two 
to  three  weeks.  In  other 
cases  in  which  slight  reac- 
tions of  degeneration  are 
present,  the  disease  may  last  two  to  three  months.  In  cases  in 
which  the  faradic  contractility  is  absent  for  a  period  of  two  to  three 
months,  the  prognosis  is  uncertain  but  not  always  serious,  as  I  have  seen 
such  cases  improve  greatly  after  persistent  electrical  stimulations.  In 
grave  cases,  in  which  the  reactions  of  degeneration  are  marked  or  elec- 
trical irritability  is  entirely  absent,  recovery  may  be  only  partial  or  not 
at  all.  In  cases  with  incomplete  recovery  the  paralyzed  muscles  become 
permanently   contracted.     The   contractured   muscles  draw   the   mouth 


Fig.  131 — Right  Facial  Paralysis  showing  Involve- 
ment   ONLY  OF  THE  UPPER  FACIAL  BRANCH. 


DISEASES    OF    CRANIAL    NERVES  409 

toward  the  paralyzed  side,  the  naso-labial  fold  is  deep,  the  palpebral 
fissure  is  small,  so  that  the  impression  may  be  formed  that  the  paralysis 
is  on  the  normal  side.  It  is  then  sufficient  to  observe  the  patient  in  volun- 
tary acts  (laughing,  showing  teeth,  etc.),  to  determine  which  half  of  the 
face  was  originally  paralyzed.  In  facial  palsy  of  nuclear  origin  the  prog- 
nosis is  very  serious. 

Diagnosis. — The  deviation  of  the  face,  the  lowering  of  the  angle  of  the 
mouth,  the  disappearance  of  wrinkles,  the  inability  to  close  the  eye  and  to 
wrinkle  the  forehead  are  sufficient  symptoms  to  recognize  a  peripheral 
facial  palsy. 

Cerebral  Facial  Palsy. — A  cerebral  lesion  producing  a  hemiplegia  quite 
frequently  involves  also  the  face.  This  is  due  to  an  involvement  of  the 
fibers  passing  from  the  cortical  center  of  the  face  through  the  internal 
capsule  together  with  those  from  the  arm  and  leg  centers  and  joining  the 
nucleus  of  the  facial  nerve  in  the  pons  on  the  opposite  side  (see  Anatomy). 
Therefore  a  facial  palsy  of  cerebral  origin  almost  always  accompanies 
a  hemiplegia. 

In  addition  to  this  the  following  symptoms  are  characteristic  of  cerebral 
facial  paralysis :  (a)  preservation  of  the  musculus  frontalis  and  orbicularis 
palpebrarum.  This  is  possibly  due  to  the  fact  that  the  upper  branch  of 
the  facial  nerve  receives  its  fibers  from  a  different  tract  (perhaps  from  the 
third  nerve  at  the  lowest  level  of  the  peduncles  or  sixth  nerve)  or  else  it 
is  innervated  by  both  hemispheres;  (b)  normal  electrical  reactions  of  the 
affected  muscles;  (c)  absence  of  atrophy;  (d)  paralysis  of  the  voluntary 
movements  of  the  mouth  and  preservation  of  emotional  movements. 
To  sum  up,  in  cerebral  facial  paralysis  only  the  lower  part  of  the  face  is 
affected,  while  in  peripheral  or  nuclear  palsy  the  upper  and  the  lower  halves 
of  the  face  are  paralyzed. 

An  isolated  involvement  of  the  upper  branch  of  the  facial  nerves  has 
occasionally  been  reported.  The  accompanying  photograph  shows  a  paral- 
ysis of  the  right  musculus  frontalis  and  orbicularis  palpebrarum  without 
any  other  central  or  peripheral  symptom  (Fig.  131).  In  this  case  there 
was  also  a  dilatation  and  a  sluggish  fight  reaction  of  the  pupil  on  the 
same  side.  The  condition  of  the  pupil  points  to  a  physiological  connec- 
tion of  the  seventh  with  the  third  nerve.  Indeed  Mendel  long  ago  main- 
tained the  idea  that  the  upper  branch  of  the  facial  nerve  takes  its  origin 
in  the  lower  part  of  the  oculo-motor  nucleus  (Neurol.  Centralblatt.,  1898). 
He  found  atrophy  of  the  cells  in  this  nucleus  after  section  of  the  upper 
facial  nerve  in  rabbits.  Besides,  in  bulbar  palsy  and  amyotrophic  lateral 
sclerosis  there  is  a  comparative  escape  of  the  muscles  of  the  upper  face, 
while  those  of  the  lower  are  involved.     In  spite  of  this  evidence,  not  all 


41 0  DISEASES    OE    CRANIAL    NERVES 

are  agreed  on  a  separate  origin  of  the  upper  branch  of  the  facial  nerve. 
The  majority  of  writers  believe  that  it  arises  from  the  dorsal  part  of  the 
facial  nucleus. 

In  nuclear  palsy  there  is  frequently  a  simultaneous  involvement  of  the 
seventh  and  the  sixth  nerve  in  view  of  the  contiguity  of  the  nuclei,  also 
involvement  of  the  limbs. 

Double  Peripheral  Facial  Palsy  (facial  diplegia)  may  simulate  bulbar 
and  pseudo-bulbar  palsies.  In  the  first  there  are  usually  present  abnormal 
phenomena  of  deglutition,  phonation  and  respiration,  in  the  other  there 
is  an  apoplectic  seizure  with  cerebral  phenomena.  In  facial  diplegia 
the  expression  is  characteristic.  The  mouth  is  open,  saliva  is  continuously 
running,  also  tears  are  on  the  cheeks.  The  eyelids  and  the  entire  muscu- 
lature of  the  face  are  immobile.  The  voice  is  nasal;  mastication  is  diffi- 
cult. Facial  diplegia  is  due  most  of  the  time  to  a  bilateral  lesion  of  the 
petrous  bones  or  basal  meningitis,  also  to  bilateral  tumors  of  the  cerebello- 
pontine angle.     In  the  latter  case  there  is  also  bilateral  deafness. 

In  peripheral  facial  palsy  it  is  important  to  determine  the  seat  of  the 
lesion.     It  can  be  facilitated  by  the  following  special  symptoms: 

(a)  A  lesion  between  the  stylo-mastoid  forearm  and  the  periphery 
will  involve  only  the  facial  muscles,  (b)  A  lesion  in  the  Fallopian  canal 
below  the  geniculate  ganglion  will  involve  also  the  taste  on  the  anterior 
two-thirds  of  the  tongue  (chorda  tympani)  and  the  hearing,  (c)  A  lesion 
at  the  level  of  the  geniculate  ganglion  and  above  it  will  involve  the  palate 
in  addition  to  all  the  other  symptoms  except  the  taste,  (d)  A  lesion  at 
the  base  of  the  brain  will  involve  simultaneously  the  eighth  nerve,  and 
deafness  will  appear  at  the  same  time  as  paralysis  of  the  face. 

Treatment. — Removal  of  the  cause  is  the  first  indication.  Tumors, 
abscesses,  otitis  media  will  be  treated  accordingly.  When  a  syphilitic 
history  is  present,  iodides  and  mercury  will  be  given.  Cases  which  are 
due  to  cold  and  which  come  under  observation  at  or  shortly  after  the  onset 
of  the  palsy  should  be  treated  at  first  with  vesicants  or  bloodletting  back 
of  the  ear.  Warm  applications,  sedatives  or  coal-tar  products  (salicylates, 
aspirin,  phenacetin)  will  relieve  pain.  At  the  end  of  ten  days  after  the 
acute  symptoms  have  subsided,  galvanic  applications  to  the  paralyzed 
muscles  must  be  instituted.  At  first  they  will  be  daily,  but  later  every 
other  day  ten  to  fifteen  minutes'  application  is  sufficient.  At  the  end  of 
a  few  weeks  faradism  can  be  substituted  for  the  galvanism.  Massage 
is  a  good  adjuvant  to  electricity.  A  daily  rub  for  five  to  ten  minutes  will 
be  useful.  Secondary  contractures  are  not  easily  remedied,  although  the 
treatment  should  be  kept  up  indefinitely.  I  have  obtained  good  results 
in  very  old  cases  after  a  prolonged  treatment.     The  surgical  treatment 


DISEASES    OF    CRANIAL    NERVES  411 

consists  of  anastomosis  of  the  facial  nerve  with  the  spinal  or  with  the  hypo- 
glossus.     Satisfactory  results  have  been  obtained  from  both  procedures. 

F.  Paralysis  of  the  Eighth  Nerve  (Auditory). 

Etiology. — Middle  ear  or  labyrinth  diseases,  trauma,  caries  and 
other  diseases  of  the  bones  at  the  base,  meningitis  (especially  its  cerebro- 
spinal form),  syphilis — may  all  be  accompanied  by  a  neuritis  of  the 
eighth  nerve.  A  basal  disease  rarely  involves  only  the  eighth  nerve, 
the  facial  nerve  also  suffers.  Degeneration  and  atrophy  of  the  eighth 
nerve  are  occasionally  observed  in  tabes,  paresis,  multiple  sclerosis.  A 
prolonged  use  of  quinine  may  produce  degeneration  of  the  acoustic 
nerve. 

Symptoms. — As  the  eighth  nerve  consists  of  two  portions  with  differ- 
ent functions   (see  Anatomy),    the  symptoms  will   differ   according   to 
whether  the  cochlear  or  the  vestibular  nerves  are  involved. 

(a)  Cochlear  Nerve  Symptoms. — The  function  of  this  nerve  is  hearing. 
Disturbances  of  hearing  will  result  from  diseases  of  this  nerve. 

Tinnitus  Aurium  is  one  of  the  symptoms.  It  is  characterized  by 
subjective  sounds,  as  ringing,  buzzing,  etc.  If  the  condition  continues, 
deafness  may  be  the  result.  In  making  a  diagnosis  it  should  be  borne 
in  mind  that  tinnitus  'may  be  caused  by  any  disease  of  the  auditory 
system  or  by  general  diseases.  Thus  anaemia,  hyperemia  and  aneurism 
of  cerebral  vessels;  otitis  externa  or  media,  cerumen  in  the  external  ear — 
are  all  apt  to  produce  peculiar  sounds. 

Deafness,  partial  or  complete,  may  be  caused  by  a  lesion  of  the  cochlear 
nerve  as  well  as  by  diseases  of  the  ear  itself.  The  differential  diagnosis 
is  based  upon  the  following  facts.  If  deafness  is  due  to  a  disease  of  the 
middle  ear,  the  tuning  fork  is  not  heard  when  kept  near  the  ear,  but 
heard  when  applied  to  the  temporal  bone.  In  a  disease  of  the  cochlear 
nerve  the  fork  is  not  heard  when  applied  to  the  bone.  In  diseases  of 
the  middle  ear  hearing  is  increased  in  a  noise  but  not  increased  in  diseases 
of  the  cochlear  nerve.  Congenital  or  hereditary  deafness  is  probably 
due  to  a  primary  atrophy  of  the  eighth  nerve. 

(b)  Vestibular  Nerve  Symptoms. — Anatomically  the  nerve  originates 
in  the  semilunar  canals  and  labyrinth.  Its  function  is  to  maintain 
equilibrium.  The  reason  of  it  lies  mainly  in  the  connections  existing 
between  the  nuclei  of  this  nerve  and  the  cerebellum,  which  is  the  chief 
organ  of  equilibrium.  An  affection  of  the  nerve,  or  of  its  nuclei,  produces 
vertigo.  The  nuclei  connecting  the  vestibular  nerve  with  the  cerebellum 
(middle  and  lateral  lobes)  are:  vestibular,  Deiter's,  and  dorsal  auditory 
(see  Anatomy). 

It  should  not  be  forgotten  that  vertigo  may  result  from  other  causes 


412  DISEASES    OF    CRANIAL   NERVES 

besides  a  lesion  of  the  vestibular.  Thus  diseases  of  the  ear,  of  the  ocular 
apparatus,  of  the  brain,  of  the  viscera,  may  be  attended  by  vertigo. 
It  may  also  be  present  in  hysteria  and  neurasthenia.  A  detailed  discus- 
sion on  the  differential  signs  in  those  various  affections  will  be  properly 
given  in  the  chapter  "Vertigo."  The  reader  is  also  referred  to  text- 
books on  ear  diseases  for  a  detailed  study  of  tinnitus  aurium  and  deafness. 

Prognosis. — It  is  unfavorable,  as  the  underlying  cause  is  a  degenera- 
tion and  atrophy  of  the  auditory  nerve. 

Treatment. — Electricity,  and  especially  the  galvanic  current,  may 
sometimes  relieve  the  tinnitus  aurium  (anode  to  the  diseased  ear),  but 
not  much  reliance  can  be  placed  on  it  or  on  medications.  Pilocarpin, 
quinine  may  sometimes  yield  some  results.  Counter-irritation  over 
the  temporal  bone  (petrous  bone)  and  strychnia  may  be  of  some  use. 
In  syphilis  iodides  and  mercury  give  good  results. 

G.  Paralysis  of  the  Ninth  Nerve  (Glossopharyngeal) . 

Etiology. — Tumors,  gummata,  aneurismus,  injuries,  thrombosis  of  the 
jugular  veins — are  the  causes  in  the  diseases  of  the  ninth  nerve.  An 
isolated  paralysis  of  this  nerve  is  rare. 

Symptoms. — Some  of  the  motor  and  sensory  fibers  of  the  ninth 
nerve  are  intimately  connected  with  those  of  the  tenth  nerve  so  that  it 
is  difficult  to  say  that  the  functions  of  the  first  depend  upon  one  or  the 
other  nerve.  However,  it  is  admitted  that  when  the  ninth  nerve  is  in- 
volved, the  following  symptoms  are  observed:  Anaesthesia  of  the  pharynx, 
palate  and  middle  ear,  ageusia  (loss  of  taste)  of  the  posterior  third  of 
the  tongue  and  gums,  difficulty  of  swallowing,  of  phonation  and  respira- 
tion  (paralysis  of  some  muscles  of  larynx,  pharynx  and  oesophagus). 

H.  Paralysis  of  the  Tenth  Nerve  (Pneumogastric) . 

Etiology. — In  alcoholic  and  diphtheritic  polyneuritis  the  tenth  nerve 
is  frequently  involved.  Various  diseases  at  the  base  of  the  brain  or 
skull  (meningitis,  tumors,  hemorrhages,  etc.),  suppuration  or  tumors 
of  the  neck  and  mediastinum,  injuries  on  the  neck,  pericarditis,  ligation 
of  the  carotids,  are  all  apt  to  injure  the  vagus.  Finally  a  neuritis  of 
rheumatic  nature  not  infrequently  involves  the  recurrent  laryngeal 
nerves  (see  Anatomy) . 

Symptoms.— Paralysis  of  the  larynx,  fauces  and  palate  are  the  most 
prominent  symptoms.  Phonation  is  disturbed.  Speech  is  nasal.  Swal- 
lowing is  only  slightly  disturbed. 

In  unilateral  paralysis  there  is  acceleration  of  the  heart-beat,  also 
irregularity  of  respiration. 

The  most  important  symptoms  are  those  concerning  the  larynx. 
This  organ  is  supplied  by  two  branches  of  the  tenth  nerve,  viz.  superior 


DISEASES    OF    CRANIAL    NERVES  413 

laryngeal  and  recurrent  laryngeal.  The  first  contains  sensory  fibers  for 
the  mucous  membrane  of  the  upper  portions  of  the  larynx  and  motor 
fibers  for  the  epiglottis  and  cricothyroid  muscle.  The  second  supplies 
all  other  muscles  and  the  mucous  membrane  of  the  larynx  below  the 
vocal  cords  and  trachea.  The  vocal  cords  are  paralyzed  and  remain 
immovable  during  phonation  and  respiration.  In  unilateral  paralysis  the 
voice  is  hoarse  and  a  deep  inspiration  produces  stridor,  there  is  no  cough. 
In  bilateral  paralysis  aphonia  and  dyspnoea  are  present,  stridor  only  on 
deep  inspiration,  and  there  is  no  cough. 

In  nuclear  involvement  (which  occurs  in  bulbar  palsies)  of  the  vagus 
respiratory  and  laryngeal  palsies  are  manifested  by  rapid  heart  action  and 
Cheyne-Stokes'  respiration.  There  is  usually  an  associated  paralysis  of 
the  muscles  of  the  soft  palate  and  of  pharynx.  Paralysis  of  the  palate 
is  manifested  in  disturbed  phonation. 

Prognosis.- — It  is  always  grave.  A  nuclear  palsy  of  the  vagus  caused 
by  a  hemorrhage  or  embolism  may  produce  sudden  death. 

Treatment.— In  syphilis  mercury  and  iodides  may  be  of  benefit.  In 
palsy  of  the  vagus,  caused  by  alcohol,  stimulants  are  urgent:  alcohol  should 
be  then  freely  administered.  Iodides  are  useful  in  lead  laryngeal  paral- 
ysis.    Electricity  applied  externally  to  the  larynx  may  be  of  benefit. 

Paralysis  of  the  recurrent  laryngeal  nerve  deserves  special  mention. 
The  paralysis  may  be  total  or  partial.  In  the  first  case  the  vocal  cord  is 
in  an  intermediary  position  and  all  the  muscles  supplied  by  the  nerves  are 
paralyzed.  In  the  second  case  the  vocal,  cord  is  immovable  in  median 
position,  which  is  due  to  paralysis  of  the  abductor,  viz.  posterior  crico- 
arytenoid muscle.  The  partial  paralysis  is  more  frequent  than  the  total,  on 
the  left  side  more  than  on  the  right  and  oftener  in  men  than  in  women. 

The  affections  in  which  involvement  of  this  nerve  is  observed  are :  (a) 
diseases  of  the  thyroid  gland,  goiter  especially,  in  which  case  it  is  due  to  com-, 
pression.  Thyroidectomy  is  not  rarely  followed  by  paralysis  of  the  recurrent 
nerve.  In  this  case  it  may  be  due  to  pulling,  pinching  with  forceps  during 
the  operation  or  else  by  a  cicatrix  during  the  healing  process.  Most  fre- 
quently the  paralysis  is  partial.  If  it  is  unilateral,  the  sound  cord  may 
compensate  the  impairment  of  the  affected  cord,  but  in  bilateral 
paralysis  there  are  aphonia  and  respiratory  disturbances,  (b)  Aneurism 
of  the  Aorta  and  Aortitis. — The  relation  of  the  left  recurrent  nerve  to  the 
concave  portion  of  the  arch  of  the  aorta  explains  the  frequency  of  the 
nerve  paralysis  in  aneurism.  Not  only  compression  of  the  aneurism,  but 
also  an  inflammatory  process  spreading  from  the  outer  wall  of  the  aorta 
to  the  nerve  is  the  cause  of  paralysis  of  the  latter.  The  right  recurrent 
nerve  is  very  rarely  affected.     More  rarely  is  observed  a  bilateral  in- 


414  DISEASES    OF    CRANIAL   NERVES 

volvement.  (c)  Cancer  of  the  oesophagus  causes  the  disease  through 
compression  of  the  peritracheo-laryngeal  glands  which  are  frequently 
infiltrated  in  cancer  of  oesophagus,  (d)  In  cancer  of  the  lungs  and  (e) 
in  pulmonary  tuberculosis,  the  infiltrated  pretracheobronchial  glands 
being  in  contact  with  the  recurrent  laryngeal  nerve,  produce  its  paraly- 
sis, (f)  Mitral  stenosis  has  occasionally  been  the  cause  of  paralysis  of  the 
left  recurrent  nerve,  (g)  In  Tabes  paralysis  of  this  nerve  has  been  ob- 
served and  it  is  probably  due  to  a  peripheral  neuritis  (Dejerine  and  Petren) . 
(h)  Toxic  and  infectious  causes:  lead,  arsenic,  typhoid  fever,  diphtheria. 
(i)  Finally  there  may  be  a  primary  neuritis  the  cause  of  which  cannot  be 
ascertained. 

I.  Paralysis  of  the  Eleventh  Nerve  (Spinal  Accessory). 

Etiology. — Colds,  trauma  of  the  neck,  diseases  of  the  cervical  vertebras, 
abscesses  of  the  neck,  tumors  in  the  same  vicinity,  cervical  myelitis — are  all 
apt  to  compress,  irritate  or  destroy  the  eleventh  nerve.  Finally  a  primary 
neuritis  is  also  possible. 

Symptoms. — The  inner  branch  of  the  eleventh  nerve  is  in  connection 
with  the  tenth  nerve  and  controls  phonation  movements  of  the  larynx. 
We  will  therefore  be  concerned  here  exclusively  with  the  external  branch. 
The  latter  innervates  the  muscles  sterno-cleido-mastoid  and  trapezius. 
In  unilateral  paralysis  of  the  first  muscle  that  of  the  opposite  side  will 
bend  the  head  towards  it  and  the  face  will  turn  towards  the  paralyzed 
muscle.  The  deviation  will  be  only  slight,  as  the  sterno-cleido-mastoid 
muscle  is,  besides  the  eleventh,  also  supplied  by  second  and  third  cervical 
nerves.  When  the  paralysis  is  bilateral,  the  head  cannot  be  held  straight, 
but  falls  backward. 

When  the  trapezius  is  involved,  a  marked  deformity  ensues.  The 
function  of  this  muscle  is  to  turn  the  head  backward  and  elevate  the 
shoulder.  In  case  of  paralysis,  the  serratus  not  being  counteracted 
rotates  the  scapula  so  as  to  project  its  inner  angle  upward,  and  when  the 
arm  is  brought  forward,  the  scapula  is  no  more  held  against  the  thorax, 
but  projects.     In  bilateral  paralysis  shrugging  of  the  shoulder  is  impaired. 

In  advanced  cases  atrophy,  reactions  of  degeneration,  also  contrac- 
tures develop.  The  deformity  is  then  pronounced.  Trapezius  and 
sternomastoid  muscles  may  be  paralyzed  in  the  course  of  progressive 
muscular  atrophy,  in  syringomyelia,  in  tabes,  when  the  cells  of  the  an- 
terior cornua  of  the  upper  portion  of  the  cord  become  involved. 

Prognosis  depends  upon  the  cause.  Syphilis  has  the  most  favorable 
outlook. 

Treatment  consists  of  electricity  and  massage.  In  cases  of  tumors 
an  operation  is  indicated.     Old  cases  with  contractures  will  be  treated 


DISEASES    OF    SPINAL    NERVES  415 

by  tenotomy  or  orthopedic  appliances.  In  syphilitic  cases  mercury  and 
iodides  should  be  given. 

J.  Paralysis  of  the  Twelfth  Nerve  (Hypoglossus). 

Etiology.- — Diseases  at  the  base  of  the  skull  (tumors,  hemorrhages, 
caries),  aneurism  of  vertebral  arteries,  dislocation  of  upper  cervical 
vertebrae,  direct  injury — are  all  causes  of  paralysis. 

Symptoms.— In  unilateral  paralysis  the  tongue  is  deviated  towards 
the  side  of  the  paralysis.  This  can  be  explained  by  the  action  of  the 
genio-hyo-glossus  of  the  normal  side.  The  affected  half  of  the  tongue 
is  atrophied,  flabby  and  wrinkled.  Fibrillary  contractions  are  marked. 
Reactions  of  degeneration  are  present.  Hemiatrophy  of  the  tongue  does 
not  interfere  to  a  great  extent  with  mastication  and  deglutition.  These 
two  acts  are  decidedly  impaired  in  total  paralysis  .of  the  tongue:  the 
latter  lies  motionless  in  the  mouth.  Articulation  of  words  is  indistinct 
in  unilateral  paralysis,  but  marked  in  bilateral.  In  nuclear  disease 
paralysis  of  the  tongue  is  associated  with  paralysis  of  the  lips. 

Prognosis. — It  depends  upon  the  cause.  Recovery  has  been  observed 
in  syphilitic  cases. 

Treatment. — The  original  cause  must  be  removed  whenever  it  is 
possible.  Electricity  may  be  of  some  use.  Mercury  and  iodides  should 
be  given,  when  syphilis  is  suspected. 

II.  Paralysis  of  Spinal  Nerves 

A.  Upper  Cervical  Nerves 

The  third  and  fourth  cervical  roots  give  off  filaments  to  form  a  nerve 
the  function  of  which  is  of  extreme  importance.  This  is  the  phrenic 
nerve. 

Paralysis  of  the  Phrenic  Nerve 

Etiology. — Cervical  or  mediastinal  tumors,  infectious  diseases  (diph- 
theria, etc.), intoxications,  toxic  polyneuritis,  (alcohol,  lead,  carbon  monox- 
ide), Potts'  disease,  dislocations  or  fractures  of  the  vertebras,  finally  diseases 
of'  the  upper  cervical  cord  (hemorrhages,  tumors,  myelitis,  poliomyelitis, 
pachymeningitis,  syphilitic  meningitis) — are  all  causes  of  phrenic  nerve 
palsy.  Occasionally  the  phrenic  nerve  is  involved  in  tabes.  Finally  in 
pleurisy  and  peritonitis  the  branches  of  the  nerve  distributed  in  the 
diaphragm  may  become  affected. 

Symptoms, — As  the  function  of  this  nerve  is  mainly  to  innervate  the 
diaphragm,    a   unilateral  paralysis   of  it  will  produce  disturbances   of 


416  DISEASES    OF    SPINAL    NERVES 

respiration.  Dyspnoea  is  the  chief  symptom.  It  is  marked  upon  the 
least  effort.  The  acts  of  coughing,  expectoration,  defecation  and  even 
talking  increase  the  respiratory  disorder.  In  severe  cases,  when  the  paraly- 
sis is  bilateral,  the  dyspnoea  is  pronounced  even  when  the  patient  is  at 
rest.  The  patient  is  then  threatened  with  asphyxia.  On  inspection 
the  diaphragmatic  phenomenon  of  Litten  is  noticeable.  It  consists  of 
an  epigastric  depression  during  the  act  of  inspiration  and  of  a  protrusion 
in  the  act  of  expiration.  Pressure  over  the  scalene  muscles  or  between 
the  lower  insertions  of  sterno-mastoid  muscle  produces  pain. 

Course,  Prognosis. — The  disease  is  serious  and  the  danger  lies  in 
asphyxia  or  when  bronchitis  or  pneumonia  develop.  In  the  latter  case 
the  respiratory  trouble  is  increased.  The  prognosis  is  graver  when  the 
paralysis  depends  upon  a  cord  lesion  than  upon  neuritis.  It  is  also  very 
grave  in  diphtheritic  and  quite  serious  in  alcoholic  neuritis.  In  making 
a  diagnosis  it  should  be  borne  in  mind  that  hysteria  sometimes  simulates 
phrenic  paralysis.  The  special  stigmata,  the  sudden  onset  of  the  palsy 
after  an  emotion  will  decide  the  diagnosis  of  the  hysteria. 

Treatment. — Removal  of  the  cause  as  promptly  as  possible  is  the  main 
indication.  CouDter-irritation  applied  to  the  space  between  the  bellies 
of  the  sterno-mastoid  muscle  may  be  of  benefit.  Strychnia  and  electricity 
are  advisable.  Asphyxia  can  be  relieved  by  inhalation  of  oxygen.  Anti- 
syphilitic  treatment  should  be  instituted  when  specific  disease  is  suspected. 

B.  Paralysis  of  the  Lower  Cervical  Nerves 

Paralysis  of  the  Brachial  Plexus 

The  last  four  cervical  roots  and  the  first  dorsal  constitute  the  brachial 
plexus.  A  lesion  of  the  roots  cannot  be  differentiated  from  that  of  the 
plexus  itself.  In  the  description  that  follows  we  will  be  concerned  with 
various  palsies  of  the  upper  limb  caused  by  a  lesion  extending  from  the  point 
of  emergency  of  the  roots  from  the  cord  till  the  point  where  the  individual 
nerves  leave  the  plexus. 

Etiology.— Injury  of  the  shoulder,  dislocation  or  fracture  of  the  head 
of  the  humerus  and  of  the  clavicle,  operative  procedures  in  the  same  region, 
obstetrical  manceuvers,  forceps,  forced  reduction  of  dislocated  humerus, 
forced  extension  or  abduction  of  the  arm,  heavy  weights  on  the  shoulders 
(stone  or  hod  carriers)- — are  all  traumatic  causes.  Tumors  or  abscesses 
in  the  vicinity  of  the  plexus,  diseases  of  the  bony  tissue  (caries,  exostosis 
of  the  vertebrae  or  clavicle),  localized  meningitis,  finally  a  neuritis  of  rheu- 
matic, toxic  and  infectious  nature  are  the  non-traumatic  causes.  Cervical 
rib  may  be  also  the  cause  of  the  inferior  type  of  brachial  palsy  (see  below). 


DISEASES    OF    SPINAL    NERVES  417 

Symptoms. — The  paralysis  of  the  upper  extremity  is  total  when  the 
lesion  affects  the  entire  plexus;  partial,  when  only  a  portion  of  the  plexus 
is  involved. 

Total  Paralysis. — It  is  rare.  It  is  usually  preceded  by  a  numbness 
or  pain  of  neuralgic  character.  The  entire  upper  limb  is  paralyzed. 
Abduction,  adduction,  flexion,  extension,  movements  of  the  hands  are 
all  abolished.  The  electrical  reactions  are  at  first  only  diminished, 
but  later  show  RD.  Atrophy  develops  rapidly  and  appears  first  in  the 
upper  portions  of  the  limb.  The  skin  is  cold  and  its  secretions  are  dimin- 
ished. Sensations  are  abolished.  There  is  complete  anaesthesia  to 
touch,  pain  and  temperature  over  the  entire  arm  except  on  the  internal 
aspect  of  the  arm  which  is  also  innervated  by  the  second  and  third  dorsal 
nerves. 

As  the  first  dorsal  nerve  enters  into  the  formation  of  the  brachial 
plexus,  in  addition  to  the  above  symptoms  there  will  be  also  oculo- 
pupillary  manifestations,  viz.  myosis,  narrowness  of  the  palpebral  fissure. 

The  course  and  prognosis  depend  upon  the  intensity  of  the  involve- 
ment. While  recovery  is  possible,  however  in  the  majority  of  cases 
partial  paralysis  and  atrophy  remain.  Retraction  of  the  tendons  results 
in  deformities  of  the  arm  or  hand.  Most  frequently  the  paralysis  is 
at  first  total,  then  gradually  becomes  limited  to  one  or  two  nerves, 
chiefly  musculo-spiral  and  circumflex. 

Partial  Paralysis. — It  presents  two  main  types :  Superior  and  inferior. 

(a)  Superior  Type  (Erb's  paralysis). — The  following  muscles  of  the 
shoulder  and  arm  are  involved:  deltoid,  biceps,  brachialis  anticus,  supi- 
nator longus,  supinator  brevis,  supra-  and  infraspinatus  and  the  clavicular 
end  of  the  pectoralis  magnus.  These  ^muscles  are  controlled  by  that 
part  of  the  brachial  plexus  which  takes  its  fibers  from  the  fifth  and  sixth 
cervical  roots. 

The  disturbed  function  consists  of  an  inability  to  flex,  supinate  and 
abduct  the  arm.  The  latter  is  in  a  state  of  extension.  The  forearm 
and  hand  are  pronated.  Atrophy  with  partial  or  complete  RD  develop 
rapidly.  Sensations  as  a  rule  are  not  disturbed,  but  there  may  be  anaes- 
thesia in  the  areas  of  distribution  of  the  circumflex  and  musculo-cutane- 
ous  nerves. 

The  symptoms  just  enumerated  may  present  variations  as  to  their 
intensity.     Finally,  the  paralysis  may  be  bilateral. 

The  upper  type  of  brachial  palsy  is  particularly  frequent  at  birth. 

It  is  the  so-called  Birth  or  Obstetrical  Palsy  (Duchenne).     It  is  almost 

always  due  to  instrumental  delivery  (forceps)   or  difficult  labor.     The 

finger  or  tenaculum  introduced  to  facilitate  the  delivery,  also  the  forceps 
27 


4i8 


DISEASES    OF    SPINAL    NERVES 


may  press  directly  on  the  shoulder  and  the  brachial  plexus.  In  breech 
presentation  it  is  very  frequent.  The  symptoms,  course  and  prognosis 
are  as  described  above  with  this  difference,  however,  that  the  prognosis 
is  more  favorable.     Complete  recovery  is  not  infrequent. 

(&)  Inferior  Type  (Klumpke) . — Anatomically  it  is  due  to  a  lesion  of 
the  seventh,  eighth  cervical  root  and  the  first  dorsal.  It  is  characterized 
by  paralysis  of  the  flexors  of  the  hand,  thenar,  hypothenar  and  interossei 
muscles.  The  hand  is  claw-like.  Atrophy  sets  in  early.  Sensory 
disturbances  consist  of  anaesthesia  on  the 
inner  half  of  the  forearm  and  hand  (ulnar 
nerve) ;  sometimes  it  extends  to  the  area  of 
distribution  of  the  median  nerve.  Pain  is 
not  infrequently  present. 

As  the  first  dorsal  nerve  is  involved,  oculo- 
pupillary  symptoms  are  present.  They  are: 
myosis,  sluggish  contraction  of  the  pupil,  re- 
traction of  the  eyeballs  and  narrowness  of  the 
palpebral  fissure. 

(c)  Complex  Type. — The  two  types  of 
partial  paralysis  of  the  brachial  plexus  do  not 
always  present  themselves  as  accurately  local- 
ized as  described  above.  Sometimes  the 
lesion  extends  to  neighboring  roots.  In  other 
cases  there  may  be  a  combination  of  both 
types,  but  each  or  only  one  of  them  is  par- 
tially involved.  In  such  cases  the  sympto- 
matology is  naturally  complex. 

Treatment. — It  is  that  of  neuritis  in  general  (see  this  chapter).  Re- 
moval of  the  cause  is  the  main  indication.  Pain  is  relieved  by  appro- 
priate means.  Electricity  and  massage  constitute  the  most  important 
part  of  the  treatment. 

Paralysis  of  Individual  Nerves  of  the  Brachial  Plexus. 

(a)  Long  Thoracic  Nerve  (from  fifth  and  sixth  cervical  roots). — It 
supplies  the  serratus  muscle. 

Etiology. — Injury  or  prolonged  pressure  on  the  neck  (in  carrying 
heavy  weights  on  the  shoulder),  gunshot  wounds  of  the  neck,  continuous 
elevation  of  the  arm  (as  in  plastering  ceilings) ,  infectious  diseases  (diph- 
theria, typhoid  fever,  grippe),  finally  exposure  to  cold — are  the  causes. 

Symptoms. — Pain  may  or  may  not  precede  the  onset  of  paralysis 
of  the  serratus  muscle.  The  function  of  the  latter  is  to  rotate  the  scapula 
when  the  arm  is  put  forwards,  also  to  hold  the  scapula  against  the  thorax. 


Fig.  132. — Showing  Position 
or  the  Arm  in  Birth  Palsy. 


DISEASES    OF    SPINAL    NERVES 


419 


In  case  of  paralysis  of  the  muscle  the  arm  cannot  be  raised  above  a 
horizontal  position  and  in  the  attempt  to  put  the  arm  forwards  the  inner 
border  of  the  scapula  becomes  separated  from  the  thorax  (wing-like). 

The  Course  and  Prognosis  depend  upon  the  cause.  The  disease 
lasts  many  months.  Traumatic  cases  are  the  most  unpromising.  Rest 
of  the  arm  (sling)  is  advisable.  Massage  and  electricity  should  be 
instituted  as  early  as  possible. 

(b)  Supra-Scapular  Nerve  (from  fifth,  sixth,  also  fourth  roots). — It 
supplies  the  supra-  and  infraspinatus  muscles. 


Fig.  133.  Fig.  134. 

Figs.  133,  134. — Paralysis  of  Serratus  Magnus  Causing  Displacement  of  Scapula 

when  the  Arm  is  Held  Forward.     (From  Nour.  Iconogr.  de  la  Salpetriere.) 

Etiology — Injury  to  the  shoulder,  dislocation  of  the  humerus,  pressure 
upon  the  shoulder  are  the  causes  of  palsy  of  this  nerve. 

Symptoms. — Pain  may  be  present  at  the  onset.  The  function  of  the 
infraspinatus  muscle  is  to  rotate  the  humerus  outward.  Its  paralysis  abol- 
ishes this  function,  and  this  is  seen  in  the  act  of  writing  and  sewing.  The 
patient  thus  affected  is  unable  to  move  the  pen  on  the  paper.  Paralysis 
of  the  supraspinatus  interferes  with  the  act  of  raising  the  arm,  also  permits 
the  head  of  the  humerus  to  lose  its  firm  position  in  the  glenoid  cavity. 
The  muscles  eventually  atrophy.  Pain  is  frequent  and  anaesthesia  over 
the  scapula  is  present. 

Treatment. — Same  as  above. 

(c)  Circumflex  Nerve  (from  fifth  and  sixth  cervical  roots). — It  supplies 
the  deltoid  and  teres  minor  muscles;  also  the  skin  over  the  deltoid  and  the 
articulation. 


420 


DISEASES    OF    SPINAL    NERVES 


Etiology. — Injury,  dislocation  and  contusion  of  the  shoulder,  compres- 
sion (crutch),  lead  poisoning,  grippe,  diabetes,  finally  exposure  to  cold 
are  the  usual  causes  of  palsy. 

Symptoms. — The  function  of  the  deltoid  muscle  is  to  elevate  the  arm. 
Its  paralysis  abolishes  almost  all  power  of  raising  the  arm.  The  atrophy 
of  the  muscle  changes  the  shape  of  the  shoulder.  Anaesthesia  is  present 
over  the  skin  covering  the  muscle.  Adhesions  may  form  in  the  joint 
and  produce  ankylosis.  Reactions  of  degeneration  appear  quite  early, 
except  in  cases  of  contusion. 


Fig.  135. — Paralysis  or  Serratus  Magnus.     Position  of  Scapula  when  Arm  is  Abducted. 
Further  Elevation  of  Arm  is  Impossible.     (Starr.) 

(d)  Musculo-Cutaneous  Nerve  (from  fifth,  sixth,  also  seventh  roots). — 
It  supplies  the  biceps  and  brachialis  anticus,  also  the  skin  over  the  radial 
side  of  the  forearm. 

Etiology.- — Trauma,  pressure,  dislocation  or  fracture  of  the  head  of 
the  humerus  are  the  usual  causes  of  palsy. 

Symptoms. — Paralysis  of  the  flexors  of  the  forearm  is  the  chief  symp- 
tom. It  is  particulary  marked  when  the  arm  is  supinated.  Atrophy 
and  RD.  develop  rapidly.  There  is  also  anaesthesia  on  the  outer  side  of  the 
forearm. 

(e)  Musculo-Spiral  Nerve  (from  sixth,  seventh  and  eighth  roots). — 
It  supplies  the  triceps,  supinators  and  extensors  of  the  hands  and  fingers; 
the  skin  of  the  lower  half  of  the  arm,  of  the  back  of  the  forearm,  also  of 
the  dorsum  of  the  thumb  and  of  the  first  three  fingers. 

Etiology. — The  superficial  position  of  the  nerve  renders  it  very  sus- 
ceptible to  injuries.    Trauma  is  therefore  the  most  frequent  of  all  causes 


DISEASES    OF    SPINAL    NERVES 


421 


of  musculo- spiral  paralysis.  Compression,  acute  or  chronic,  is  a  common 
occurrence  It  develops  during  a  profound  sleep,  especially  in  an  intoxi- 
cated state,  when  the  head  rests  on  the  arm  a  number  of  hours  (Saturday 
night  paralysis).  It  also  occurs  during  a  deep  narcosis  when  the  arm  is 
held  against  a  hard  support.  The  superficial  position  of  the  nerve  in 
the  axilla  is  the  cause  of  its  palsy  when  the  patient  walks  on  crutches 
(crutch  palsy).  Violent  muscular  exercise,  fracture  of  the  humerus  may 
also  produce  a  musculo-spiral  palsy.  Infectious  diseases,  intoxications 
(alcohol,  lead,  arsenic)  are  predisposing  causes.  Potain  mentions  hypo- 
dermic injections  made  close  to  the  nerve  as  an  occasional  cause  of  its 
paralysis. 


Fig.  136. — Position  of  the  Hand  in  Musculo-Spiral  Palsy.     (Oppenheim.) 

Symptoms. — The  onset  is  usually  sudden  It  may  be  preceded  by  some 
paraesthetic  disturbances  (tingling,  etc.).  As  the  main  function  of  the 
nerve  is  extension,  its  paralysis  will  place  the  hand  in  a  special  position. 
The  latter  is  flexed,  adducted  and  semi-pronated;  the  fingers  are  equally 
flexed  and  the  thumb  is  adducted.  There  is  a  gradation  of  paralysis 
of  extension  in  the  fingers,  most  pronounced  in  the  fourth  finger  and  least 
marked  in  the  first  (Gowers).  Extension  of  the  wrist  is  impossible 
(wrist-drop).     The  forearm  is  also  flexed  and  pronated. 

The  state  of  sensations  depends  upon  the  seat  of  the  damage  dene  to 
the  nerve.  In  the  majority  of  cases  the  injury  occurs  near  the  musculo- 
spiral  groove;  the  sensibility  is  here  only  exceptionally  disturbed.  When 
the  nerve  is  damaged  above  that  level,  anaesthesia  will  be  present  over 
the  area  supplied  by  the  sensory  filaments  of  the  nerve  (see  above). 

The  paralyzed  muscles  gradually  undergo  atrophy  and  changes  in  elec- 
trical irritability.  The  latter  is  at  first  diminished,  but  later  presents  reac- 
tions of  degeneration. 

Course,  Termination,  Prognosis. — They  depend  to  a  large  extent 
upon  the  cause.     Crutch  palsy  improves  rapidly  when  the  crutches  are 


42  2  DISEASES    OF    SPINAL    NERVES 

abandoned.  The  usual  cases  of  pressure  palsy  improve  and  recover. 
When  the  nerve  is  severely  injured  by  fracture,  dislocation  or  contusion, 
recoven*  may  also  fellow,  but  slowly.  The  prognosis  is  almost  in  direct 
relationship  to  the  electrical  condition  of  the  muscles.  Diminution  of 
response  has  a  favorable  outlook,  RD. — is  unfavorable.  When  the  nerve 
is  completely  severed,  restoration  of  function  will  be  possible  only  after 
a  reunion  of  the  stumps. 

Treatment. — Removal  of  the  cause  in  cases  of  compression  is  the  first 
indication.  As  alcoholic  individuals  are  particularly  apt  to  suffer,  they 
should  be  insisted  upon  abstaining  from  alcohol.  When  the  nerve  is 
completely  severed,  suturing  of  the  two  ends  is  necessary.  Electricity 
and  massage  are  the  usual  procedures  for  restoring  the  function  of  the 
paralyzed  muscles.  They  should  be  instituted  as  early  as  possible. 
Mechanical  apparatus  have  been  recommended  for  the  relief  of  flexion 
of  the  wrist  and  fingers;  their  object  is  to  place  the  latter  in  extension. 

(J)  Ulnar  Nerve  (from  eighth  cervical  and  first  dorsal  roots). — It 
supplies  the  flexor  carpi  ulnaris,  the  inner  portion  of  the  flexor  digitorum 
profundus,  the  interossei  of  the  hand,  the  muscles  of  the  little  finger, 
the  palmaris  brevis,  the  last  two  lumbricales,  hypothenar,  the  adductor 
pollicis.  It  gives  off  sensory  fibers  to  the  integument  of  the  hypothenar 
to  the  front  of  the  fifth  finger  and  half  of  the  fourth  finger,  to  the  back 
of  the  fifth,  fourth  and  half  of  the  third  finger. 

Etiology. — The  superficial  position  of  the  nerve  at  the  elbow  and  wrist 
predisposes  it  to  injury.  Pressure  more  or  less  prolonged,  laceration  of  the 
nerve,  fracture  and  dislocation  at  the  level  of  the  elbow,  diseases  of  the 
olecranon  or  internal  condyle  of  the  humerus,  forced  flexion,  occupations 
requiring  a  prolonged  flexion,  especially  in  alcoholic  or  cachectic  individuals 
are  all  causes  of  paralysis  of  the  ulnar  nerve.  Overextension  of  the  arm 
during  surgical  operations  is  apt  to  produce  palsy  of  the  ulnar  nerve.  It 
is  probably  due  to  stretching  of  the  nerve  in  the  axilla.  In  sleep  a  similar 
phenomenon  may  be  observed  when  the  individual  sleeps  with  arms  ele- 
vated, abducted  and  behind  the  head.  Such  a  case  I  placed  on  record 
{Old  Dominion  Journal  of  Med.  and  Surg.,  1909). 

Symptoms. — Paralysis  of  the  above  enumerated  muscles  will  be  present. 
The  most  characteristic  disturbance  is  the  paralysis  of  the  interossei. 
The  normal  function  of  the  latter  consists  of  flexion  of  the  first  and  extension 
of  the  last  two  phalanges,  also  adduction  and  abduction  of  the  fingers. 
When  paralysis  occurs,  the  fingers  show  an  exaggerated  extension  of  the 
first  and  flexion  of  the  last  two  phalanges.  This  is  the  claw-like  hand. 
The  condition,  however,  is  only  partial;  more  pronounced  for  the  fourth 
and  fifth  fingers  than  for  the  index  and  middle  fingers,  because  the  latter 


DISEASES    OF    SPINAL    NERVES  423 

have  their  lumbricales  (median  nerve)  preserved.  Adduction  of  the  thumb, 
abduction  and  adduction  of  the  fingers,  lateral  movements  of  the  little 
finger  are  impossible.  The  hand  is  slightly  deviated  toward  the  radial 
side. 

Sensory  Disturbances  are  habitually  present.  Besides  pain  and  hy- 
peresthesia there  may  be  hypsesthesia  or  anaesthesia.  The  latter  will  be 
present  over  the  internal  surface  of  the  hand  (palm  and  dorsum) ,  little  finger 


Fig.  137.  Fig.  138. 

Figs.  137,  138. — Showing  Sensory  Loss  and  Abnormal  Position  in  Injuries  of  Ulnar 

Nerve.     (Bowlby.) 

inner  half  of  the  fourth  finger,  internal  and  dorsal  surface  of  the  first 
phalanx  of  the  third  finger. 

Atrophy  and  reactions  of  degeneration  develop  some  time  after  the 
onset  of  the  palsy. 

Course,  Prognosis. — In  slight  traumata  the  termination  is  favorable 
after  a  few  weeks'  duration.  In  lacerations  or  severance  the  prognosis  is 
unfavorable  as  to  recovery  of  function. 

Treatment. — Same  as  in  musculo-spiral  nerve  palsy. 

R.  Hunt  described  in  1908  an  occupation  neuritis  due  to  an  involve- 
ment of  the  deep  palmar  branch  of  the  ulnar  nerve.  This  nerve  is  purely 
motor  and  innervates  the  following  intrinsic  muscles  of  the  hand:  those 
forming  the  hypothenar  eminence,  also  some  of  the  thenar  region,  viz. 
abductor  pollicis  and  inner  head  of  the  flexor  brevis  pollicis.  It  innervates 
as  well  the  palmar  and  dorsal  interossei  and  the  two  inner  lumbricales. 

The  symptoms  are:  atrophic  paralysis  of  all  the  intrinsic  muscles  of  the 


424 


DISEASES    OE    SPINAL    NERVES 


hand  innervated  by  the  ulnar  nerve;  reactions  of  degeneration;  total 
absence  of  objective  sensory  disturbances  in  the  ulnar  nerve  distribution. 

The  lesion,  which  is  usually  due  to  compression,  is  below  the  point  where 
the  sensory  branch  is  given  off  and  before  the  deep  branch  breaks  up  into 
muscular  branches.  Hunt  observed  the  neuritis  in  a  jeweler,  machinist, 
brass  polisher. 

H.  Gessler  in  1896  (Mediz.  Corresp.-blatt.,  Wurtemberg,  Bd.  LXVI)  de- 
scribed a  type  of  muscular  atrophy  of  the  hand  in  gold  polishers,  which 
resembles  in  many  respects  that  of  Hunt  with  this  difference  that  there  was 
paresthesia  in  the  distribution  of  the  ulnar  nerve. 

(g)  Median  Nerve  (from  sixth,  seventh,  eighth  cervical  and  first 
thoracic). — It  supplies  the  superficial  anterior  (except  the  flexor  carpi 


Fig.  139.  Fig.  140. 

Figs.  139,  140. — Showing   Areas   of  Sensory  Losses   in  Injuries  of  Median  Nerve. 

(Bowlby.) 
Horizontal  lines,  total  anaesthesia;  vertical  lines,  partial  anaesthesia. 

ulnaris)  and  the  deep  muscles  of  the  forearm  (except  the  inner  half  of  the 
flexor  digitorum  communis).  In  the  hand  it  supplies  the  opponens 
pollicis,  the  flexors  of  the  thumb,  the  abductor  brevis  and  the  two  outer 
lumbricales.  It  also  gives  off  sensory  branches  to  the  radial  part  of  the 
palm,  inner  part  of  the  thenar  and  the  palmar  aspect  of  the  first  three 
fingers  and  the  radial  side  of  the  fourth  finger,  also  the  dorsum  of  the 
last  two  phalanges  of  the  thumb  and  first  three  fingers. 

Etiology. — Trauma  (blows,  cuts,  fracture)  in  the  lower  part  of  the  fore- 
arm, compression  (tumor,  callus,  Esmarch's  bandage,  etc.),  violent  muscular 


DISEASES    OF    SPINAL    NERVES  425 

efforts  are  the  most  frequent  causes.  Certain  occupations  (tinners,  joiners 
cigarmakers,  milkers)  may  sometimes  involve  the  muscles  innervated  by 
the  median  nerve.  Finally  infectious  diseases  may  occasionally  cause  a 
neuritis  of  the  median  nerve. 

Symptoms. — Paralysis  of  the  muscles  enumerated  above  will  give 
the  hand  a  peculiar  attitude.  The  position  of  the  fingers  is  quite  char- 
acteristic. The  last  two  phalanges  are  held  in  forced  extension  by  the 
interossei  (ulnar  nerve)  more  on  the  radial  than  on  the  ulnar  side;  the 
thumb  is  extended,  approached  to  the  index  and  its  opposition  is  impossi- 
ble. The  wrist  is  in  extension  and  slightly  abducted.  Pronation  of  the 
hand  is  impossible. 

Sensory  Disturbances  are  usually  present.  They  may  consist  of 
pain,  hyperesthesia  and  especially  anaesthesia.  The  latter  extends  over 
the  two  outer  thirds  of  the  palm,  the  palmar  surface  of  the  first  three 
fingers  and  radial  side  of  the  fourth  finger,  the  dorsal  aspect  of  the  last 
two  phalanges  of  the  thumbs,  second  and  third  fingers  and  radial  side 
of  the  fourth  finger. 

Atrophy  with  reactions  of  degenerations  appear  early.  Trophic  and 
vasomotor  disturbances  of  the  skin  are  quite  frequent  (herpes,  glossy 
skin,  loss  of  nails,  hyperhidrosis,  cyanosis). 

Course,  Prognosis  and  Treatment  are  identical  to  those  of  the  ulnar 
nerve. 

C.  Paralysis  of  Lumbo-sacral  Nerves 

Paralysis  of  the  Nerves  of  the  Lower  Extremities 

(a)  Anterior  Crural  Nerve  (from  second,  third  and  fourth  lumbar 
roots). — It  supplies  the  ileo-psoas,  extensor  cruris  quadriceps,  sartorius 
and  partly  the  pectineus  and  the  middle  adductor  muscles.  Its  cutaneous 
branches  are  distributed  over  the  antero-internal  surface  of  the  thigh 
(lower  two-thirds)  and  internal  aspects  of  the  leg  and  foot. 

Etiology. — Palsy  of  the  crural  nerve  is  rare.  Diseases  of  the  vertebras, 
pelvis  and  femur,  dislocation  of  the  hip-joint,  psoas  abscess,  hernia, 
aneurism,  trauma  in  the  groin  or  thigh  are  the  main  causes.  It  may 
become  paralyzed  in  infectious  diseases  (typhoid  fever,  diphtheria)  also 
in  alcoholism.  Finally,  in  anterior  poliomyelitis  the  muscles  supplied 
by  the  anterior  crural  nerve  are  especially  involved. 

Symptoms. — The  distribution  of  the  nerve  indicates  that  its  paralysis 
affects  especially  the  extensors  of  the  knee  and  flexors  of  the  thigh.  Paraly- 
sis of  these  muscles  will  cause  a  loss  of  the  knee-jerk. 

Sensory  Disturbances  consist  of  hypaesthesia  or  anaesthesia  over  the 


426  DISEASES    OF    SPINAL    NERVES 

area  of  distribution  of  the  cutaneous  fibers  (see  above).     Pain  is  frequent. 
Atrophy  and  reactions  of  degeneration  appear  early. 

(b)  Obturator  Nerve  (from  second,  third  and  fourth  lumbar  roots). — 
It  supplies  the  gracilis,  adductor  longus  et  brevis.  Its  sensory  fibers  are 
distributed  over  the  inner  side  of  the  thigh  and  inner  and  upper  third  of 
the  leg. 

Etiology.- — Obturator  hernia,  compression  by  tumors  or  in  difficult 
labor  are  the  usual  causes. 

Symptoms. — Adduction  of  the  thigh  and  its  outward  rotation  are 
affected.  The  patient  is  unable  to  cross  and  approach  the  thighs,  also  to 
turn  the  foot  inward  and  outward.  Loss  of  sensation  will  be  present  over 
the  area  of  distribution  of  the  cutaneous  filaments. 

Atrophy  and  reactions  of  degeneration  develop  early. 

(c)  Paralysis  of  the  Gluteal  Nerves  (from  the  lumbar  and  sacral 
plexus) . — They  supply  the  gluteal  muscles,  the  pyrif ormis  and  the  tensor 
fasciae  lata?. 

Etiology. — Diseases  of  sacrum  and  pelvis,  fractures,  tumors  are  the 
usual  causes.     The  disease  is  rare. 

Symptoms. — Paralysis  of  the  above  muscles  produces  loss  of  rotation, 
abduction,  extension  and  flexion  of  the  thigh.  Station,  gait  and  climbing 
are  difficult. 

Atrophy  develops  rapidly. 

(d)  External  Cutaneous  Nerve  (from  second  and  third  lumbar  nerves). 
Symptoms. — As  it  supplies  the  outer  aspect  of  the  thigh,  its  paralysis 

causes  a  difficulty  of  walking  and  standing  (see  alsoMeralgia  paraesthetica). 

(e)  Paralysis  of  the  Sciatic  Nerve  (from  Sacral  Plexus). 

Etiology. — Pressure  by  tumors  of  the  pelvis  or  femur  or  fractures,  by  the 
head  of  the  foetus  in  difficult  labor,  by  forceps  in  instrumental  delivery,  neu- 
ritis of  toxic  (alcohol,  lead,  arsenic)  or  infectious  nature,  septic  processes  of 
the  pelvis — are  all  causes  of  total  paralysis  of  the  sciatic  nerve.  Complete 
palsy  is  rare.  More  frequently  are  met  partial  palsies  of  the  sciatic  nerve, 
viz.  paralysis  of  the  peroneal  nerves.  In  the  latter  case  infection  and 
intoxications  play  a  prominent  etiological  role.  Puerperal  neuritis  has  a 
special  predilection  for  the  external  popliteal  nerve.  Fracture  of  the 
fibula,  forcible  extension,  occupations  requiring  a  stooping  position  are 
also  causes  of  peroneal  nerve  palsy. 

Symptoms. — In  total  paralysis  of  the  sciatic  nerve  (which  is  exception- 
ally rare)  the  foot,  the  leg,  also  the  flexors  of  the  leg  situated  on  the  thigh 
are  all  completely  powerless.  The  motility  of  the  entire  limb  is  abolished 
with  the  exception  of  extension  of  the  leg,  which  is  controlled  by  the 
crural  nerve  (see  above).     The  leg  is  held  rigid.     The  gait  is  possible  to 


DISEASES    OF    SPINAL    NERVES  427 

a  certain  extent  because  of  the  integrity  of  the  extensor  muscles  (crural 
nerve).  Atrophy  and  anaesthesia  are  common.  Sometimes  trophic 
disturbances,  such  as  herpes,  etc.,  are  present. 

In  paralysis  of  the  external  popliteal  nerve  the  following  muscles  are 
involved : 

(a)  The  peroneal,  the  function  of  which  is  to  extend,  abduct  and  rotate 
(externally)  the  foot;  (b)  tibialis  anticus  whose  function  is  to  flex,  adduct 
and  rotate  (internally)  the  foot;  (c)  extensor  communis  digitorum  which 
extends  the  first  phalanges  of  the  toes;  (d)  extensor  of  the  great  toe  which 
extends  its  first  phalanx. 

When  these  muscles  are  paralyzed,  the  action  of  the  posterior  muscles 
predominates;  dorsal  flexion  of  the  foot  is  impossible.  The  latter  is  in  a 
state  of  foot-drop  and  of  equino-varus.  The  gait  is  of  steppage  character 
(see  Multiple  Neuritis) .  In  an  advanced  stage  contractures  place  the  foot 
and  its  constituents  in  a  fixed  abnormal  position.  Sensations  are  usually 
diminished.  There  may  also  be  total  anaesthesia.  The  latter  is  distrib- 
uted over  the  antero-external  surface  of  the  leg  and  the  dorsum  of  the  foot 
and  toes.  Trophic  and  vaso-motor  disturbances,  atrophy  of  the  muscles, 
changes  in  the  electrical  reactions  are  the  same  as  in  any  other  nerve 
palsy. 

Special  mention  must  be  made  of  paralysis  of  the  external  popliteal 
nerve  occurring  during  protracted  labor.  This  nerve  originates  from  the 
lumbo-sacral  trunk  which  is  formed  by  the  union  of  the  smallest  part  of 
the  fourth  and  the  entire  fifth  lumbar  nerves.  The  trunk  at  its  formation 
is  situated  on  the  ala  of  the  sacrum  under  cover  of  the  psoas.  It  descends 
into  the  pelvis  and  passes  over  the  brim  of  the  true  pelvis.  It  stands  to 
reason  that  a  protracted  labor  with  head  pressing  unduly  against  the  brim 
of  the  pelvis,  or  else  forceps,  are  apt  to  injure  the  exposed  lumbo-sacral  cord, 
and  therefore,  the  peroneal  nerve.  Besides  this  grave  form  of  peroneal  neu- 
ritis there  is  also  a  slight  form  of  neuritis  occurring  in  puerperium  and  which 
is  usually  transient.  It  is  probably  tonic  in  nature.  In  both  forms  there 
is  pain  on  pressure,  paretic  state  of  the  muscles,  spontaneous  pain,  ob- 
jective sensory  disturbances  and  sometimes  oedema  with  cyanosis  of  the 
limb. 

The  diagnosis  should  be  made  between  neuritis  of  the  peroneal  nerve 
and  puerperal  phlebitis.  Failure  of  recognizing  the  true  condition  may 
lead  to  very  grave  errors  of  treatment.  When  for  example  a  neuritis  is 
taken  for  phlebitis,  it  means  a  strict  and  prolonged  immobilization  of  the 
limb.  This  will  lead  eventually  to  muscular  atrophy,  loss  of  power  in  the 
limb,  trophic  disturbances,  otherwise  speaking  to  a  protracted  infirmity. 
When  on  the  other  hand  a  phlebitis  is  taken  for  a  neuritis,  it  means 


428  RADICULITIS 

mobilization  of  the  limb,  electrization  and  massage  of  the  muscles.  In 
both  cases  the  respective  procedures  are  dangerous.  A  very  careful  ex- 
amination for  characteristic  symptoms  of  each  of  these  affections  will  en- 
able one  to  avoid  errors. 

When  the  internal  popliteal  nerve  is  involved  the  following  muscles 
muscles  are  paralyzed:  (a)  Triceps  suras  (which  normally  produces  plantar 
flexion) ;  (b)  tibialis  posticus,  which  is  an  extensor,  adductor  and  rotator 
(internally)  of  the  foot;  (c)  flexors  of  the  toes,  viz.  of  their  first  phalanges; 
(b)  interossei  (which  normally  flex  the  first  phalanges  and  extend  the 
others) . 

Paralysis  of  these  muscles  leads  to  dorsal  extension  of  the  first  phalanges 
of  the  toes  and  plantar  flexion  of  the  last  two  phalanges  (claw-like),  to 
loss  of  plantar  flexion.  Rising  on  the  tips  of  the  toes  is  impossible  and 
walking  is  difficult. 

Sensory  Disturbances  (hypaesthesia  or  anaesthesia)  are  present  on  the 
plantar  surface  of  the  foot  and  on  the  postero-internal  surface  of  the  leg. 

RADICULITIS. 

Under  this  term  is  understood  a  primary  inflammation  of  the  roots 
of  nerves.  Secondarily  the  nerve  roots  may  be  involved  in  lesions  of 
peripheral  nerves  or  of  the  spinal  cord  (poliomyelitis,  syringomyelia,  etc.) 
and  in  traumatic  injuries.  But  radiculitis  means  a  primary  infection 
or  toxic  inflammation  of  the  roots  and  their  coverings. 

Symptoms. — The  onset  may  be  rapid  or  slow.  The  first  is  rare;  it 
may  occur  in  the  course  of  cerebro-spinal  or  spinal  meningitis.  The  second, 
which  is  the  usual  onset,  consists  of  slowly  but  progressively  developing 
manifestations.  As  a  type  the  most  frequent  form  of  radiculitis  will 
be  described  here,  viz.  cervico-brachial  radiculitis  of  tertiary  syphilis. 
Gradually  and  without  apparent  cause  various  paraesthesias  and  pain 
develop  in  the  cervical  region.  Pain  is  continuous  but  is  interrupted  by 
paroxysms  of  a  violent  character.  The  latter  are  brought  on  by  an 
effort,  cough  or  sneezing.  The  exacerbations  make  the  patient  scream 
and  Dejerine  with  others  described  this  phenomenon  "sign  of  sneezing" 
as  a  reliable  symptom  of  the  disease.  The  pain  radiates  along  the  course 
of  the  nerve-trunks.  Pressure  provokes  pain.  It  can  be  elicited  at  the 
level  of  the  sixth  transverse  process  (Erb's  point).  Gradually  the  nervous 
filaments  undergo  degeneration  and  destruction  and  the  hyperaesthesia 
is  replaced  by  anaesthesia ;  pain  then  disappears.  Not  only  the  skin 
but  also  muscles,  bones  and  joints  have  lost  their  sensibility.  Perception 
of  position  and  stereognostic  sense  are  abolished.     For  this  reason  ataxia 


RADICULITIS  429 

becomes  evident.  Whether  the  loss  of  sensation  is  complete  or  incomplete, 
it  is  always  radicular  in  distribution  and  the  latter  is  characteristic  of  the 
disease. 

When  the  lesion  affects  also  the  motor  roots  as  well  as  the  sensory 
there  will  be  present  also  motor  and  trophic  symptoms  in  addition  to 
sensory.  Paresis  or  paralysis,  atrophy  of  the  muscles  with  reactions 
of  degeneration  gradually  make  their  appearance.  When  atrophy  affects 
only  a  certain  group  of  muscles,  the  antagonistic  muscles  through  their 
overaction  will  produce  deformities  of  the  limbs.  The  tendon  reflexes 
are  at  first  diminished,  but  later  disappear.  Trophic  disturbances  are 
not  infrequent.  Herpes  zoster  may  accompany  a  radiculitis  of  the  sen- 
sory roots.  A  very  important  diagnostic  sign  we  find  in  the  cerebro-spinal 
fluid.  As  the  usual  etiological  factor  is  syphilis  (also  tuberculosis), 
a  more  or  less  marked  lymphocytosis  is  found. 

Similar  to  neuritis  and  neuralgia  there  are  also  radiculitis  and  radic- 
ulalgia.  In  the  latter  the  lesion  consists  only  of  irritation,  but  not  of 
destruction.  The  pain  is  very  severe,  paroxysmal  in  type  and  hyper- 
esthesia of  radicular  distribution  is  present.  Chipault  and  Lefur  described 
a  neuralgia  of  the  eighth,  ninth,  and  tenth  dorsal  roots  which  manifested 
itself  as  a  tic  douloureux  of  the  abdomen.  Neuralgia  of  the  roots  may  be 
the  initial  stage  of  radiculitis. 

Forms  of  Radiculitis 

Cervico-brachial. — It  was  described  above  as  a  type.  If  the  eighth 
cervical  and  first  dorsal  are  involved,  oculo-pupillary  symptoms  will  be 
also  observed,  viz.  myosis,  retraction  of  the  eye  globe  and  narrowness 
of  the  palpebral  fissure. 

Dorsal. — Vaso-motor  disturbances,  such  as  herpes  zoster,  will  be 
observed.  Such  was  the  case  of  Dejerine  and  Thomas  in  which  the  erup- 
tion was  strictly  limited  to  the  area  of  the  right  eighth  dorsal  root.  Oppen- 
heim  observed  paralysis  of  the  abdominal  muscles  and  loss  of  their  reflexes, 
meteorism,  pain  and  sensory  disturbances  in  lesions  of  the  eighth  and  ninth 
dorsal  roots. 

Lumbar.- — It  is  quite  frequent.  It  presents  the  picture  of  neuritis  of 
the  anterior  crural  nerve  (see  this  chapter). 

Lumbo-sacral. — Its  requisition  is  very  important.  It  constitutes  the 
so-called  "radicular  sciatica."  The  differential  diagnosis  between  it 
and  the  classical  sciatica  is  as  follows:  in  the  latter  the  objective  sensory 
disturbances  (hyperesthesia  or  anesthesia)  are  distributed  irregularly. 
In  the  former  the  sensory  disturbance  will  be  present  in  a  longitudinal 


43  O  NEURALGIA 

form  regularly  distributed  and  running  parallel  with  the  axis  of  the  limb. 
Besides,  the  pain  is  spontaneous  and  brought  on  not  exclusively  on  motion, 
also  it  is  very  persistent  and  present  in  regions  others  than  the  ischio- 
sacral  notch  (see  my  monograph  in  /.  Amer.  Med.  Ass'n,  1910). 

Sacro-coccygeal. — The  radiculitis  of  the  cauda-equina  is  accompanied 
by  disturbances  of  the  function  of  the  sphincter  and  genitalia. 

Etiology. — Any  cause  that  is  capable  to  produce  an  inflammation  or 
degeneration  of  the  roots  in  their  intra-  or  extra-dural  course  can  be  con- 
sidered as  an  etiological  factor.  Tumors,  hemorrhages,  cold  abscess, 
aneurism — are  all  apt  to  bring  on  a  mechanical  irritation  of  the  tissue 
surrounding  the  root  or  else  to  disturb  the  circulation  and  nutrition  of  the 
latter.  Tuberculosis  (in  Pott's  disease)  and  cancer  (of  the  vertebra?) 
are  almost  always  the  causes  of  radiculitis.  Hypertension  of  the  cerebro- 
spinal fluid  in  tumors  of  brain  and  in  tabes  have  also  been  considered  as 
causes.  More  important  etiological  factor  is  an  infectious  process.  Here 
an  inflammation  of  the  meninges  occurs  first  and  of  the  root  subsequently. 
After  syphilis,  tuberculosis  is  the  most  important  factor  (pachymenin- 
gitis, leptomeningitis).  Toxic  (lead)  and  infectious  agents  have  a  special 
predilection  for  nerve-roots. 

Prognosis  is  quite  serious.     In  syphilitic  cases  it  is  most  favorable. 

Treatment. — It  depends  on  the  cause.  Operations  (laminectomy,  re- 
section of  posterior  roots)  are  advisable  only  as  a  last  resort.  (For  more 
details  consult  the  thesis  of  Paris  by  Camus,  1908.) 

NEURALGIA  (IN  GENERAL) 

Definition  and  Nature. — Under  this  term  is  understood  a  paroxysmal 
pain  over  nerve  trunks  and  their  branches.  Neuralgia  is  not  a  morbid 
entity,  but  only  a  symptom,  as  it  may  accompany  any  affection  of  the 
nerves.  Pain  in  the  nerve  may  be  also  present  when  no  lesion  of  the 
latter  is  evident.  A  sharp  distinction  between  neuralgia  and  neuritis 
cannot  be  established.  The  fact  is  that  in  a  number  of  cases  of  recent 
neuralgia  degenerative  changes  have  been  found  in  the  nerves.  From 
my  personal  pathological  studies  (New  York  Med.  Jour.,  July  21,  1906) 
it  can  be  seen  that  the  occurrence  of  degeneration  of  the  peripheral  nerves 
is  frequent  in  neuralgia,  that  neuralgia  is  probably  a  primary  neuritis  and 
that  changes  in  the  walls  of  the  blood  vessels  play  a  certain  role  in  the 
causation  of  the  degenerative  condition  of  the  nerves. 

Etiology. — From  the  foregoing  remarks  it  is  evident  that  the  causes  of 
neuritis  (see  this  chapter)  will  be  also  those  of  neuralgia. 

Besides  neuritis,  neuralgia  may  be  due  to  exhaustion  caused  by  de- 


NEURALGIA 


43 1 


bilitating  and  protracted  diseases,  such  as  cancer,  anaemia,  to  infectious 
diseases  (grippe,  typhoid  fever,  malaria),  to  syphilis,  to  intoxications 
(lead,  arsenic,  etc.).  Exposure  to  cold  is  a  frequent  factor.  Overwork 
and  great  excitement,  errors  of  ocular  refraction,  often  provoke  neuralgic 
pains.  In  the  so-called  reflex-neuralgias  irritation  of  remote  organs 
(uterus,  testicles,  etc.)  produces  a  neuralgia  of  the  face  or  elsewhere. 

Among  the  predisposing  causes  should  be  mentioned  the  neuroses 
(hysteria,  neurasthenia),  gout,  rheumatism,  diabetes,  tuberculosis. 

Symptoms.— The  chief  symptom  is  pain.  Its  seat  is  along  the  nerve. 
Generally  it  is  manifested  in  violent  paroxysmal  attacks  between  which 
the  patient  is  free  from  pain,  but  in  some  cases  a  slight  pain  persists  in 


Fig.  141. — Neuralgia  of  Three  Months'  Duration.     Degenerated  Nerve -bundles  in 
the  Immediate  Vicinity  of  Thickened  Blood  Vessel.     (Original.) 


the  intervals.  The  paroxysms  may  last  from  a  few  seconds  to  several 
minutes;  they  may  recur  several  times  in  one  hour.  The  pain  may  be 
tolerable  or  extremely  violent,  be  confined  to  one  or  several  spots  on  the 
skin  or  else  spread  from  an  initial  point  to  several  ramifications  of  the 
nerve.  It  appears  spontaneously  or  brought  on  by  the  least  touch, 
motion,  cold  air,  mastication,  etc. 

A  characteristic  symptom  of  neuralgia  is  found  in  the  tender  spots. 
They  are  very  small  areas  situated  over  the  course  of  the  nerve  and  its 
branches;  they  are  extremely  tender  upon  slight  pressure. 

In  addition  to  these  two  constant  symptoms  there  are  others  that  occur 
more  or  less  frequently.  They  are  sensory,  motor,  vaso-motor,  trophic 
and  secretory. 


43  2  NEURALGIA 

Sensory. — They  may  be  hyperaesthesia  or  anaesthesia  over  the  area  of 
distribution  of  the  affected  nerve. 

Motor. — Twitching  during  the  paroxysms  (in  tic  douloureux)  or  else 
spasmodic  contractions  (painful  cramps)  are  sometimes  observed. 

Secretory. — They  are  ptyalism,  hyperhidrosis,  watering  of  the  eyes. 

Vaso -motor. — Pallor  or  redness  of  the  skin  is  sometimes  observed 
during  the  paroxysms;  angioneurotic  oedema  has  been  seen  in  association 
with  neuralgic  paroxysms. 

Trophic  Disturbances  accompanying  neuralgia  are:  oedema  and  herpes. 

Neuralgia  of  long  duration  may  affect  the  general  health.  The  patient 
is  depressed,  irritable,  loses  his  appetite,  cannot  sleep  and  finally  begins 
to  lose  in  weight. 

Course,  Duration,  Prognosis. — Neuralgia  is  usually  of  long  duration. 
The  paroxysms  may  occur  at  regular  or  irregular  intervals.  It  may  after 
a  certain  time  disappear  completely  or  else  become  chronic.  In  cases  of 
malarial  origin  the  neuralgia  presents  a  special  feature:  it  may  substitute  a 
malarial  attack  or  accompany  it,  viz.  occur  at  certain  intervals.  Mala- 
rial neuralgia  affects  mainly  the  fifth  and  the  sciatic  nerves.  In  grippe 
the  supra-orbital  nerve  is  most  frequently  affected. 

Neuralgia  of  diabetic  origin  has  a  special  predilection  for  the  sciatic 
nerve. 

The  course  and  prognosis  depend  upon  the  cause.  Neuralgia  caused 
by  neuritis  is  serious,  because  it  recurs  easily  and  readily  becomes  chronic. 
Malarial  and  syphilitic  neuralgias  are  usually  amenable  to  treatment. 
The  outlook  is  bad  when  there  is  a  constitutional  unfavorable  basis  (tuber- 
culosis, diabetes,  etc.). 

Diagnosis.— Pain  along  the  course  of  the  nerve,  its  tendency  to  radia- 
tion, its  remittence  or  intermittence,  finally  the  tender  points  are  all  suf- 
ficiently characteristic  symptoms  for  making  a  diagnosis  of  neuralgia. 

Sharp  pain  may  occur  also  in  tabes  (tabetic  crises),  but  in  the  latter  it 
does  not  follow  the  course  of  a  nerve  trunk  and  the  tender  points  are  ab- 
sent.    It  is  present  in  tumors  located  in  the  vicinity  of  the  spinal  roots. 

In  neuritis  pain  is  usually  present,  but  it  is  continuous,  while  in  neu- 
ralgia it  is  paroxysmal.  Neuritis  will  be  also  recognized  by  motor,  sensory 
and  trophic  disturbances,  which  are  absent  in  neuralgia. 

Treatment.— (i)  Relief  from  pain  and  (2)  removal  of  the  cause  of 
neuralgia  are  the  chief  indications.  The  pain  can  be  relieved  by  sedatives. 
Morphine  will  give  almost  immediate  relief,  but  its  repeated  administration 
is  to  be  feared  in  view  of  the  habit  which  is  easily  acquired.  Coal-tar 
products  (aspirin,  sodium  salicylate,  phenacetin,  etc.)  without  or  with 
small  doses  of  codein  has  given  me  satisfactory  results.     Local  counter- 


NEURALGIA  433 

irritation  (cantharides)  has  given  me  excellent  results  in  a  number  of  cases 
where  a  host  of  internal  medications  have  failed.  Malarial  and  grippal 
cases  need  an  energetic  treatment  with  quinine.  Syphilic  cases  require  mer- 
cury and  iodides  or  salvarsan.  In  very  obstinate  cases,  even  without  a 
syphilitic  history,  the  specific  treatment  should  be  tried. 

Good  results  have  been  obtained  by  me  from  Strychnia  in  gradually 
increasing  doses.  In  one  case  of  obstinate  cervical  neuralgia  in  a  middle- 
aged  woman,  when  all  possible  medications  had  been  tried  during  a  period 
of  two  years  and  operative  procedures  had  been  almost  decided  upon, 
strychnia  was  given  as  a  last  trial  and  curiously  enough  the  pain  dis- 
appeared completely. 

Thyroid  extract  has  also  given  me  very  satisfactory  results  in  four  cases 
when  everything  else  failed. 

Electricity  has  given  some  relief  to  a  very  limited  number  of  my 
patients.  Galvanism  and  sometimes  faradism  as  well  as  static  electricity 
had  been  used  by  me  in  those  cases.  Care  should  be  taken  to  apply  weak 
currents  (of  12-15  milliamperes)  to  the  affected  nerves,  especially  in  facial 
neuralgia. 

The  general  nutrition,  a  constitutional  diathesis,  a  neuropathic  state, 
must  be  taken  special  care  of.  Appropriate  hygiene  and  diet,  proper  and 
regular  elimination  (bowels,  kidney),  hydrotherapy,  massage,  regular 
mode  of  living,  avoidance  of  stimulants  (alcoholic  beverages),  reduction  of 
obesity  if  it  exists,  administration  of  arsenic  and  iron  in  cases  of  anaemia, 
a  special  diet  in  diabetes,  exercises  for  those  who  lead  a  sedentary  life  and 
on  the  contrary  much  rest  for  those  whose  occupation  is  exhausting — -all 
these  points  should  invariably  be  taken  into  consideration  in  treating  a 
case  of  neuralgia. 

Injections  of  alcohol  into  the  nerve  trunks  in  facial  neuralgia  at  the 
points  of  their  exit  in  the  bony  foramina  has  proven  to  be  a  very  useful 
procedure.  Five  minims  of  80  per  cent,  of  alcohol  should  be  injected 
without  a  preliminary  local  anaesthetic.  The  injection  can  be  repeated 
every  few  days.  In  the  majority  of  cases  the  pain  disappears  immediately 
after  the  injection.  Burning  and  swelling  of  the  surrounding  tissue  ap- 
pear rapidly  and  persist  for  several  days,  but  disappear  without  leaving 
any  trace.  For  supraorbital  neuralgia  injections  are  made  in  the  supra- 
orbital notch,  which  is  easily  felt.  For  the  second  branch,  alcohol  is 
injected  into  the  infraorbital  foramen.  For  the  third  branch  of  the  fifth 
nerve  injections  are  to  be  made  into  the  mental  foramen  of  the  lower  jaw. 
I  have  obtained  in  this  manner  considerable  relief  in  a  number  of  instances. 
I  have  also  observed  that  when  several  injections  into  the  infraorbital 
nerve  were  not  successful,  subsequent  injections  into  the  supraorbital 
28 


434  NEURALGIA 

nerve  gave  relief  for  weeks.  Should  the  alcoholic  injections  made  at  the 
exit  of  the  sensory  nerves  on  the  face  fail,  deep  injections  are  to  be  carried 
out.  It  must  be  mentioned  immediately  that  the  first  branch  of  the 
trigeminus  should  not  be  treated  with  deep  injections,  as  the  second, 
third,  fourth  and  sixth  cranial  nerves,  also  large  blood  vessels,  are  in  im- 
mediate vicinity  of  the  ophthalmic  branch  and  the  risk  of  their  injury  is 
very  great.  Deep  injections  into  the  second  branch  are  made  with  a 
needle  inserted  at  the  lower  border  of  the  zygoma;  0.5  cm.  behind  a 
perpendicular  running  from  the  posterior  edge  of  the  orbital  process 
of  the  malar  bone  it  is  diverted  upward;  at  a  depth  of  5  cm.  the  needle 
reaches  the  nerve  as  it  leaves  the  foramen  rotundum  to  enter  the  sphe- 
nomaxillary fossa  (Levy,  Baudouin,  Patrick).  For  the  third  branch  of 
the  trigeminus  the  needle  is  inserted  also  at  the  lower  border  of  the 
zygoma  but  2.5  cm.  in  front  of  the  anterior  root  of  the  zygoma;  it  is 
directed  backward  and  upward.  At  a  depth  of  4  cm.  it  reaches  the 
nerve.  The  directions  just  given  are  not  absolute,  as  the  anatomical 
interrelations  between  foramina  and  bony  structures  through  which  the 
nerves  pass  van*  considerably  in  some  individuals.  Considerable  experi- 
ence is  necessary  on  cadavers  before  an  attempt  can  be  made  to  perform 
a  deep  injection  on  patients.  Superficial  and  deep  injections  do  not 
always  cure  the  sufferer,  but  they  may  give  enormous  relief  for  a  very 
long  time.  In  case  alcoholic  injections  after  several  attempts  fail,  a 
radical  operation  for  removal  of  the  Gasserian  ganglion  or  for  cutting 
off  only  the  sensory  roots  may  be  resorted  to.  The  radical  operation, 
unfortunately,  does  not  remove  pain  in  every  case,  besides  being 
dangerous.  For  more  details  on  the  technic  of  deep  injections  of  alcohol 
consult  Levy  and  Baudouin  (Presse  Medicate,  1906),  also  Patrick  (/.  Am. 
Med.  Ass'n.,  191 2). 

While  alcoholic  injections  are  an  excellent  procedure  for  relieving  nerve 
pain,  nevertheless  it  must  be  borne  in  mind  that  grave  damage  may  be 
done  to  the  nerve  trunk  when  the  latter  is  placed  in  intimate  contact  with 
alcohol.  I  have  recently  shown  it  in  an  experimental  study  (Transac- 
tion of  Amer.  Xeurol.  Ass'n.,  1913). 

When  all  the  means  enumerated  above  fail  to  give  permanent  results, 
surgical  intervention  must  be  resorted  to.  The  latter  is  indicated  not  only 
in  cases  of  compression  of  a  nerve  by  a  tumor  and  abscess  or  of  any  other 
pathological  condition  in  the  vicinity  of  the  nerve,  but  also  in  all  cases  of 
neuralgia  which  ordinary  therapeutics,  including  the  injections  of  alcohol, 
fail  to  relieve.  The  operative  procedures  consist  of  nerve-stretching, 
neurectomy  and  neurorhexis.  In  the  first  the  results  are  frequently  only 
transitory.     The  second  consists  of  a  resection  of  a  certain  portion  of  the 


NEURALGIA  435 

affected  nerve.  The  first  two  methods  fail  to  give  permanent  results. 
The  third  method  is  based  upon  the  pathological  fact  that  a  violent  tearing 
out  of  a  nerve  produces  permanent  cellular  changes  in  its  nucleus  (Van 
Gehuchten).  This  procedure  has  given  by  far  better  results  than  any 
other. 

NEURALGIA  OF  INDIVIDUAL  NERVES 

A.  Neuralgia  of  the  Fifth  Nerve  (Facial  Neuralgia).  Etiology. — All 
the  causes  mentioned  in  the  chapter  on  neuralgia  in  general  are  also  factors 
in  facial  neuralgia.  Special  stress  must  be  laid  upon  cold  and  malaria, 
which  are  frequent  causes. 

Lesions  in  the  area  of  distribution  of  the  fifth  nerve,  viz.  diseases  of  the 
alveoli,  of  the  ear,  of  the  cavities  of  the  face  (nose,  frontal  sinus),  of  the  eye; 
diseases  of  the  bones  of  the  face  through  which  the  nerve  passes,  trauma- 
tism, inflammation,  are  all  local  causes  of  facial  neuralgia. 

The  disease  is  very  frequent  and  is  observed  usually  in  the  middle  age. 
It  is  more  frequent  in  females  than  in  males. 

Pathology. — The  nerve  had  been  frequently  found  in  an  inflammatory 
state  or  in  a  state  of  degeneration.  Atrophy  of  the  cells  of  the  Gasserian 
ganglion  have  been  also  observed  in  a  few  cases.  Endarteritis  has  also 
been  found. 

Symptoms. — Pain  is  the  chief  and  sometimes  the  only  symptom  of  the 
disease.  It  is  agonizing  in  character.  Its  onset  may  be  preceded  by  some 
paresthetic  disturbances.  Among  the  three  branches  of  the  nerve  the 
first  (ophthalmic)  is  most  frequently  affected. 

The  tender  points  characteristic  of  neuralgia  in  general  (see  above) 
are:  for  the  ophthalmic  branch — supraorbital  (at  the  level  of  supraorbital 
foramen) ;  nasal  (upper  part  of  the  nose) ;  for  the  supramaxillary  branch — 
infraorbital  (below  the  lower  eyelid) ;  malar ;  for  the  inferior  maxillary 
branch — temporal  (in  front  of  the  ear) ;  labial  (on  the  lower  lip) :  mental 
(place  of  exit  of  the  mental  nerve  on  the  chin). 

The  nature  of  the  pain  (tearing,  boring),  its  paroxysmal  character, 
the  tendency  to  radiation  over  the  branch  affected  and  even  beyond  its 
boundaries,  repetition  of  the  attacks  at  regular  or  irregular  intervals  and 
their  duration  (a  few  seconds) ,  the  development  of  the  pain  from  the  least 
influence  (cough,  touch,  mastication,  change  of  temperature  of  the  food, 
etc.),  are  described  in  the  chapter  on  neuralgia  in  general. 

The  less  constant  symptoms  of  facial  neuralgia  are:  secretory,  vaso- 
motor, trophic,  sensory  and  motor. 

Secretory. — During  an  attack  there  may  be  an  increased  nasal  secre- 
tion, also  watering  of  the  eye  and  salivation. 


436  NEURALGIA 

Vasomotor. — The  conjunctiva  on  the  affected  side  is  congested,  the 
eyelids  may  be  cedematous  and  the  face  flushed. 

Trophic  Disturbances  consist  chiefly  of  herpes,  which  is  not  infrequent 
in  neuralgia  of  the  first  branch  (ophthalmic  herpes  zoster).  Falling  of 
the  hair  and  hemiatrophy  of  the  face  are  occasionally  observed. 

Sensory  disorder  in  the  form  of  hyperesthesia  is  frequent  at  the  begin- 
ning, but  anaesthesia  is  observed  in  older  cases.  Photophobia  is  frequent. 
Hearing  and  taste  are  sometimes  affected. 

Motor  phenomena  are  not  rare.  They  consist  of  convulsive  move- 
ments of  the  muscles  of  the  affected  side  of  the  face  accompanying  the 
paroxysms  of  pain.  This  is  the  so-called  "Tic  douloureux."  It  is  the 
most  obstinate  form  of  facial  neuralgia. 

Course,  Termination,  Prognosis.- — In  the  majority  of  cases  neuralgia 
increases  in  intensity  at  first,  then  gradually  decreases.  It  runs  an  indefi- 
nite course.  It  may  disappear  completely,  but  usually  persists.  Recur- 
rences are  frequent.     The  prognosis  is  serious. 

Diagnosis.- — See  this  chapter  on  neuralgia  in  general. 

Treatment. — It  is  the  same  as  of  neuralgia  in  general  (see  above). 
The  surgical  treatment  consists  of  stretching  the  nerve,  section  or  excision 
of  a  portion  of  it  or  tearing  out  the  nerve  (neurorhexis)  as  near  as  possible 
the  bony  foramen  from  which  it  emerges.  The  latter  procedure  gave 
better  results  than  the  former.  The  reason  of  it  lies  in  this  observation 
(Van  Gehuchten  and  others)  that  the  cells  of  Gasserian  ganglion  corre- 
sponding to  the  torn-off  nerve  degenerate  more  readily  and  permanently. 
Finally  removal  of  the  Gasserian  ganglion  may  be  resorted  to  in  protracted 
cases  in  which  the  above  means  have  failed. 

In  treating  a  case  of  facial  neuralgia  it  should  be  borne  in  mind  that  a 
diseased  condition  of  the  nose,  ear,  eye,  teeth,  of  the  sinuses  may  be  the 
immediate  cause  of  the  pain.  An  examination  of  these  organs  is  indis- 
pensable and  a  proper  treatment  is  to  be  applied  if  indicated.  In  elderly 
individuals  I  have  obtained  very  satisfactory  results  from  nitroglycerine 
given  either  by  the  mouth  or  hypodermically. 

Favorable  results  in  treatment  of  tic  douloureux  have  been  obtained 
from  injections  of  alcohol  into  the  nerve  trunks  as  they  appear  on  the  face, 
or  into  their  roots  near  the  foramina  from  which  they  emerge.  Schlosser, 
Levy,  Baudouin,  Ostwald,  Brissaud,  Patrick,  Hecht  and  myself  have 
published  encouraging  reports.     For  details  see  page  433. 

B.  Cervico -occipital  Neuralgia.- — Under  this  name  is  understood  a  neu- 
ralgia of  the  upper  four  cervical  nerves.  In  the  majority  of  cases  it  is  the 
major  occipital  nerve  that  is  involved,  but  the  minor  occipital,  great  auric- 
ular, superficial  cervical  and  superclavicular  nerves  may  also  be  affected. 


NEURALGIA  437 

Etiology. — Cold,  trauma,  aneurism  of  the  vertebral  artery,  infectious 
diseases  (grippe,  typhoid  fever  and  others),  syphilis,  lead  poisoning,  auto- 
intoxication, gout,  diabetes,  anaemia  are  the  causes  of  occipital  neuralgia. 
It  is  also  observed  in  cervical  Potts'  disease,  cervical  adenitis,  hypertro- 
phic cervical  pachymeningitis,  localized  tumors  of  the  neck  or  of  the  cer- 
vical vertebras. 

Symptoms. — The  classical  symptoms  of  neuralgia  in  general  are 
present.  The  pain  is  unusually  severe.  It  is  continuous  with  paroxysms 
of  exacerbation.  The  slightest  movement,  cough,  sneezing,  cause  an  attack. 
The  patient  complains  of  a  sensation  of  burning,  tearing,  stabbing  in  the 
neck. 

The  tender  points  (of  Valleix)  are  present.  The  most  important  and 
constant  is  situated  midway  between  the  mastoid  process  and  the  first 
cervical  vertebrae,,  at  the  level  of  emergency  of  the  major  occipital  nerve. 
Other  points:  posterior  border  of  the  sterno-mastoid  muscle;  mastoid  proc- 
ess. In  severe  cases  there  is  also  hyperesthesia  of  the  skin  of  the  neck, 
tenderness  between  the  scapulae  and  in  the  supraclavicular  fossae,  loss  of 
hair  in  the  same  region,  myosis  on  the  affected  side. 

Prognosis. — It  is  usually  good,  but  the  disease  is  apt  to  recur.  In  one 
case  under  my  observation  it  recurred  six  times  in  four  years.  The  dura- 
tion is  uncertain.  It  may  last  several  weeks  or  months.  In  the  above  case 
the  last  attack  lasted  three  and  a  half  months. 

Diagnosis. — The  characteristic  tender  spots,  the  radiation  of  the  pain 
along  the  nerve  are  typical  enough  to  make  a  diagnosis. 

Treatment. — Cauterization,  blistering,  application  of  iodine,  blood- 
letting all  over  the  painful  area,  will  be  of  benefit.  In  the  case  mentioned 
above  the  local  treatment  was  of  no  avail.  Relief  and  subsequent  recovery 
followed  administration  of  strychnia  in  ascending  doses.  For  details 
see  Treatment  of  Neuralgia  in  general. 

C.  Brachial  Neuralgia. — This  is  a  neuralgia  of  the  brachial  plexus. 

Etiology. — Traumatism  of  the  brachial  plexus,  fractures  and  disloca- 
tions of  the  humerus,  injury  of  a  peripheral  nerve,  as  a  burn,  compression, 
bite  of  the  finger,  etc.  (reflex  neuralgia),  infectious  diseases  (grippe, 
malaria,  typhoid),  diabetes,  gout,  anaemia,  neuraesthenia,  are  all  causes  of 
this  affection. 

Symptoms. — Usually  the  pain  affects  one  tor  two  nerves  of  the  entire 
plexus.  The  ulnar  nerve  is  most  frequently  involved.  The  pain  follows 
the  course  of  the  nerve  and  is  characterized  by  paroxysmal  attacks  between 
which  the  patient  is  free  from  pain.  The  position  of  the  limb  is  character- 
istic. The  patient  holds  it  with  the  unaffected  hand  as  if  the  arm  were 
fractured. 


43^  NEURALGIA 

The  tender  spots  are  found  on  the  nerves  of  the  plexus :  for  the  musculo- 
spiral  nerve  the  groove  of  this  name  on  the  arm;  for  the  circumplex  nerve 
the  deltoid  area;  for  the  ulnar  nerve  the  elbow;  for  the  median  nerve  the 
wrist. 

Paresthesia,  hyperesthesia  or  anaesthesia  of  the  skin,  hyperhidrosis, 
are  frequently  present. 

Prognosis  is  the  same  as  in  neuralgia  in  general  but  it  depends  greatly 
on  the  cause. 

Diagnosis. — The  radiation  of  the  pain  along  the  nerve-trunks  and  the 
tender  points  are  characteristic  enough  to  recognize  the  disease.  In 
compression  of  the  cervical  spin  al  cord  pain  may  be  present  in  the 
arms,  but  there  are  always  other  symptoms,  as  paralysis,  atrophy, 
anaesthesia.  In  arthritis,  in  rheumatism,  the  pain  does  not  follow  the 
nerve-trunks. 

Treatment. — It  is  the  same  as  in  other  forms  of  neuralgia.  When  local 
treatment  and  internal  medications  are  of  no  avail,  an  operation  must 
be  resorted  to. 

D.  Intercostal  Neuralgia. 

Etiology. — The  causes  of  this  affection  are :  cold,  contusion  of  the  nerves, 
fracture  or  diseases  of  the  ribs,  compression  from  within  or  without, 
diseases  of  the  lungs  or  pleurae,  an  inflammation  of  which  is  transmitted 
to  the  nerves.  As  predisposing  factors  may  be  mentioned  cachexia, 
anaemia,  protracted  diseases,  syphilis,  gout,  chronic  rheumatism.  It 
is  more  frequently  met  with  in  women  than  in  men. 

Symptoms. — The  pain  is  usually  unilateral  and  involves  several  inter- 
costal nerves.  It  may  be  spontaneous  and  then  is  usually  deep  seated. 
Similar  to  neuralgia  in  general,  it  presents  paroxysms  which  are  extremely 
severe.  As  the  movements  of  the  thorax  provoke  pain,  the  patient 
holds  his  chest  immobile,  avoids  deep  respiration  and  speaks  with  a  low 
voice.     The  pain  frequently  radiates  to  the  back  and  to  the  arm. 

The  tender  spots  characteristic  of  neuralgia  are  here  three  in  number: 
anterior,  lateral  and  posterior.  The  first  is  at  sternocostal  articulation, 
the  second  on  the  axillary  line,  the  third  near  the  spinal  processes. 
The  skin  on  the  affected  side  is  hyperaesthetic.  Herpes  quite  frequently 
accompanies  or  follows  intercostal  neuralgia. 

Prognosis. — It  depends  upon  the  cause.  The  disease  is  usually 
persistent. 

Diagnosis. — The  above-mentioned  character  of  pain,  its  radiation  along 
the  nerves,  the  tender  points,  are  all  typical  enough  for  the  diagnosis. 
Pleurodynia  is  recognized  by  a  diffuse  pain.  In  mastodynia  the  pain  is 
in  the  breast.    Tumors  of  the  spine  or  spinal  cord  may  present  neuralgic 


NEURALGIA  439 

pain  as  the  only  symptom  for  a  long  time  before  pressure  symptoms 
make  their  appearance. 

Treatment. — The  first  indication  is  removal  of  the  cause.  General 
dyscrasic  condition,  compression,  fractures,  etc.,  must  be  taken  care  of. 
For  relief  of  pain  counter-irritation,  cauterization  and  local  applications 
of  revulsive  measures  are  of  great  benefit.  Obstinate  cases  should  be 
treated  surgically  (stretching,  resection,  tearing  out  of  the  nerves).  For 
further  details  see  Treatment  of  Neuralgia  in  general.  For  Franke's 
operation  see  page  254. 

E.  Sciatic  Neuralgia.    Sciatica. 

Etiology. — The  causes  are  local  and  general. 

Local. — Cold;  trauma  (contusion,  injury  of  the  nerve  by  a  bony  frag- 
ment) ;  prolonged  pressure  against  a  hard  object  while  sitting;  compression 
in  the  pelvis  by  tumors  and  abscesses  or  by  the  head  of  the  foetus  during 
a  protracted  labor;  inflammation  of  the  meninges  extending  to  the  roots 
of  the  sacral  plexus  and  then  to  the  sciatic  nerve. 

General. — Gout,  chronic  rheumatism,  malaria,  diabetes,  intoxications 
(lead,  carbonic  acid  gas,  alcohol),  infectious  diseases  (grippe,  typhoid  fever, 
puerperal  infection),  syphilis. 

Certain  occupations  predispose  to  sciatica.  It  has  been  observed 
quite  frequently  in  tailors  and  dressmakers. 

Sciatica  is  a  common  affection.  It  is  met  more  frequently  in  men  than 
in  women  and  particularly  between  thirty  and  fifty  years  of  age. 

Symptoms. — They  are  sensory,  motor  and  trophic,  as  the  nerve  is 
a  mixed  nerve. 

The  first  and  chief  symptom  is  pain.  It  is  localized  on  the  posterior 
aspect  of  the  limb.  It  is  continuous  and  paroxysmal.  The  pain  may 
present  various  forms  and  degrees.  In  some  cases  it  is  tearing,  burning; 
in  others  it  is  pulling.  The  paroxysmal  exacerbations  may  be  of  an  un- 
usual severity.  The  pain  may  affect  the  entire  nerve  or  ouly  segments  of 
it.  It  may  radiate  also  during  a  paroxysm  to  the  lumbar  region,  perineum 
and  genitalia. 

The  pain  is  usually  worse  in  walking  or  standing.  It  is  increased  when 
the  leg  is  extended.  Exposure  to  cold  aggravates  the  condition.  Atmos- 
pheric changes  also  increase  the  pain. 

The  tender  points  characteristic  of  neuralgia  are:  (i)  In  the  groove 
between  the  trochanter  and  the  tuber  ischii,  (2)  in  the  middle  of  the  pop- 
liteal space,  (3)  below  the  head  of  the  fibula,  (4)  behind  the  external  malle- 
olus, (5)  dorsum  of  the  foot,  (6)  Gara  {Deut.  mediz.  Wochenschr.,  191 1) 
has  recently  called  attention  to  an  abdominal  tender  spot  in  sciatica  which 
he  found  constantly  present  in  1 18  out  of  124  cases.    It  is  elicited  by  press- 


440  NEURALGIA 

ing  the  finger  into  the  abdomen  over  the  spinous  process  of  the  last 
lumbar  vertebra  on  the  affected  side.  He  locates  the  point  at  a  finger- 
breadth  below  the  umbilicus  and  two  fingerbreadths  to  the  side  of  the 
median  fine.     The  pain  from  compression  radiates  down  the  sciatic  nerve. 

The  skin  over  the  posterior  aspect  of  the  limb  is  usually  intact,  but 
sometimes  a  diminution  of  sensations  is  present.  In  grave  cases  of  sciatica 
complete  anaesthesia  has  been  observed.  Parsesthesise,  as  numbness,  cold- 
ness, tingling,  etc..  are  frequently  present. 

Motor  Disturbances  consist  of  some  loss  of  power,  fibrillary  contrac- 
tions, cramps  in  the  musculature  of  the  leg.  Gradually,  if  there  is  no 
improvement,  the  weakness  increases.  The  patient  assumes  certain  fixed 
attitudes.  In  order  to  avoid  extension  of  the  nerve  the  patient  places  the 
leg  in  flexion,  the  trunk  naturally  bends  toward  the  sound  side;  a  scoliosis 
with  the  convexity  to  the  affected  side  is  therefore  formed.  Eventually 
this  sciatic  scoliosis  becomes  permanent.  Sometimes  the  curvature  of 
the  spine  is  in  the  opposite  direction,  viz.  the  concavity  is  toward  the 
diseased  side. 

The  patellar  tendon  reflexes  are  usually  increased  on  the  diseased  side, 
although  decreased  and  normal  reflexes  have  been  observed.  The 
Achilles-tendon  reflex  is  usually  lost. 

The  trophic  disturbances  show  wasting  of  the  muscles.  There  may  be 
a  simple  flabbiness  of  the  muscles  or  a  genuine  atrophy  with  qualitative 
electrical  alterations  (RD.).  In  such  cases  the  changes  of  the  nerve  are 
profound.  The  skin  of  the  limb  is  bluish,  its  secretions  are  diminished, 
eruptions  (herpes,  acne,  erythema)  may  develop. 

Course,  Termination,  Prognosis. — In  the  mild  forms  the  pain  is  only 
paroxysmal.  Recovery  follows  in  a  few  weeks.  In  the  severe  forms  the 
pain  is  continuous,  persistent  and  lasts  months  or  years.  In  such  cases 
deformities  develop  and  the  patient  never  recovers.  Between  these  two 
extremes  there  are  many  intermediary  forms.  The  prognosis  is  particu- 
larly unfavorable,  when  atrophy  with  reactions  of  degeneration  is  present. 

The  chronic  form  is  less  hopeful  than  the  acute. 

Double  sciatica  has  an  unfavorable  prognosis,  as  it  is  usually  the  con- 
sequence of  diseases  of  the  spine  or  of  the  spinal  meninges.  It  may  also 
occur  in  diabetes. 

Diagnosis. — The  exact  seat  of  the  pain  on  the  posterior  aspect  of  the 
leg  and  the  characteristic  tender  points  are  usually  sufficient  for  making 
a  diagnosis.  There  is  another  important  diagnostic  test  which  is  de- 
cidedly pathognomonic.  It  consists  of  overextending  the  leg  of  the  patient 
when  he  is  in  a  sitting  or  lying  position;  this  manipulation  brings  on  pain. 
It  is  the  so-called  "sciatic  phenomenon"  of  Lasegue. 


NEURALGIA  441 

In  some  affections  of  the  lower  limbs  pain  may  simulate  sciatica,  but  a 
careful  examination  will  reveal  the  true  condition. 

In  muscular  rheumatism  the  pain  is  diffuse  and  the  tender  points  are 
absent. 

In  arthritis  of  the  sacro-iliac  or  hip-joints  pressure  from  outside  causes 
pain  only  at  the  level  of  the  articulation. 

In  sacro-coxalgia  Lasegue's  sign  is  also  present.  The  following 
differential  phenomena,  according  to  Gueit  (Gas.  des  hop.,  1910)  may  be  of 
use:  when  the  patient  lying  flat  on  his  back  flexes  his  legs  and  an  attempt 
is  made  to  continue  flexion  of  the  thighs,  a  sharp  pain  in  the  upper  and 
inner  portion  of  the  buttock  will  be  felt  in  sacrocoxalgia,  but  not  in 
sciatica.     In  the  latter  the  preexisting  pain  is  not  augmented. 

In  Meningo-myelitis  or  tumors  of  Cauda  equina  there  is  in  addition 
to  the  pain  in  the  limb  also  involvement  of  the  sphincters.  Besides,  the 
symptoms  are  generally  bilateral. 

An  examination  of  the  pelvic  organs  should  be  made  in  cases  of  pressure- 
sciatica,  as  a  local  unsuspected  lesion  may  be  the  direct  cause  of  the  affec- 
tion (tumor,  adhesions,  etc.).  For  radicular  sciatica  see  page  429.  In  all 
such  cases  an  X-ray  examination  must  be  made. 

Treatment. — In  cases  of  compression  by  a  tumor  or  other  elements 
removal  of  the  cause  is  the  first  indication. 

The  sciatica  itself  can  be  relieved  first  of  all  by  rest.  It  should  be  a 
rule  to  put  every  patient  suffering  from  this  disease  to  bed  in  order  to  se- 
cure absolute  rest  for  the  limb.  When  the  patient  is  restless,  the  limb 
should  be  immobilized  by  means  of  a  board  and  a  bandage.  Otherwise 
speaking,  it  should  be  treated  as  if  fractured.  Every  four  or  five  days  the 
bandage  will  be  taken  off  for  an  hour  or  so  and  a  gentle  massage  given  to  the 
leg.  In  the  majority  of  cases  absolute  immobilization  gives  relief.  Ame- 
lioration of  pain  can  be  also  obtained  from  cauterization,  counter-irrita- 
tion or  any  revulsion  over  the  course  of  the  sciatic  nerve,  from  blood-let- 
ting (leeches  or  scarification),  from  local  spraying  of  chloride  of  methyl, 
and  especially  from  hot  baths.  The  latter  can  be  given  once  or 
twice  a  day.  The  patient  sits  in  the  tub  fifteen  or  thirty  minutes. 
In  very  obstinate  cases  I  obtained  considerable  relief  from  prolonged 
baths — even  two  hours  at  a  time.  Galvanism,  the  faradic  brush  and 
static  electricity  may  sometimes  be  useful,  but  as  a  rule  these  means 
cannot  be  relied  upon  for  relieving  pain. 

Internally  the  following  drugs  can  be  given:  salicylates,  any  coal- 
tar  product,  iodides  and  bromides.  Aspirin  and  salophen  are  superior  to 
salicylates  and  in  combination  with  small  doses  of  codein  have  proven  to 
be  very  useful  in  my  hands.     Very  satisfactory  results  I  have  also  obtained 


442  NEURALGIA 

in  some  cases  from  strychnia  in  gradually  increasing  doses.  In  cases  with 
a  history  of  syphilis  or  malaria,  mercury  and  quinine  respectively  will  be 
useful. 

As  sciatica  frequently  occurs  in  gouty  and  rheumatic  individuals, 
autointoxication  probably  plays  an  important  role.  In  such  cases  avoid- 
ance of  nitrogenous  food  (meats)  is  advisable.  In  some  of  my  cases  a 
return  to  a  meat  diet  has  decidedly  aggravated  the  sciatica.  Alcoholic 
beverages  are  absolutely  forbidden,  because  alcohol  has  a  special  predi- 
lection for  peripheral  nerves.  Purgation  at  regular  intervals  is  advisable. 
In  favorable  cases  when  the  pain  has  subsided,  the  patient  is  allowed  to 
exercise  moderately  his  limb.  In  order  to  combat  the  wasting  caused  by 
disuse,  massage  should  be  then  instituted:  first  gentle  manipulation 
every  day  for  ten  to  fifteen  minutes,  later  deeper  rubbing  for  a  longer 
period  of  time.  Faradism  may  be  added  to  the  massage  in  order  to  accele- 
rate the  return  of  power  in  the  muscles. 

Recently  injections  of  alcohol  into  the  sheath  of  the  nerve  or  in  the 
immediate  vicinity  have  been  recommended,  but  some  observations 
show  that  this  procedure  may  be  followed  by  disastrous  results;  permanent 
paralysis  and  marked  trophic  disturbances  have  been  reported.  For  the 
limitation  of  alcoholic  injections  see  page  434.  The  same  remarks  can 
be  applied  to  injections  of  osmic  acid  advised  by  some  writers.  Relief 
has  also  been  obtained  from  injections  of  saline  solutions  (chloride  of 
sodium,  sulphate  of  magnesium)  or  of  plain  distilled  water  or  else  of  sterile 
air. 

A  subcutaneous  injection  of  salts  does  not  differ  from  an  ordinary 
saline  infusion.  The  dose  is  from  50  to  100  c.c.  It  has  a  mechanical  and 
analgesic  effect  on  the  nerve.  It  may  be  made  more  effective  by  adding 
cocain  or  morphine  according  to  Schleich's  formula  (feeble  solution: 
cocain,  0.01;  chloride  of  sodium,  0.20;  morphia,  0.02;  aqua,  100  grm.). 

Cordier  recommended  injections  of  sterile  air  in  the  close  vicinity  of 
the  nerve,  believing  that  the  distention  of  the  tissues  stretches  the  nerve 
filaments.  The  injection  is  made  in  the  lumbar  region,  at  the  external 
surface  of  the  thigh  and  above  the  head  of  the  fibula.  The  quantity  of 
air  injected  at  each  of  these  points  is  50  c.c.  The  injected  area  becomes 
pale.     Vigorous  massage  must  follow. 

Deep  injections  of  chloride  of  sodium  or  sulphate  of  magnesia  in  4  per 
cent,  solutions  have  given  better  results.  The  point  at  which  the  injection 
is  to  be  made  lies  at  the  junction  of  the  inner  third  with  the  two  outer 
thirds  of  a  line  drawn  between  the  sacrococcygeal  articulation  and  the 
postero-external  border  of  the  great  trochanter.  A  long  needle  is  inserted 
at  a  depth  of  4  to  9  cm.  and  40  to  60  c.c.  of  the  solution  are  injected. 


NEURALGIA  443 

These  injections  may  be  repeated  several  times.  In  one  case  I  made  six 
such  injections  at  intervals  of  five  or  six  days. 

Recently  "spinal  anaesthesia"  has  been  proposed  for  the  relief  of  sciatic 
pain.  This  procedure  (see  chapter  on  Lumbar  puncture)  which  is  em- 
ployed by  surgeons  in  operations  upon  the  lower  part  of  the  body,  gives 
excellent  results  also  in  sciatica.  Its  efficiency  is  due  to  the  effect  of  the 
injected  drug  upon  the  roots.  The  medications  used  in  these  cases  are 
cocain  or  stovain  (see  Schleich's  formula  above).  Epidural  injections 
have  given  in  some  cases  satisfactory  results.  Through  the  sacrococcygeal 
space  are  injected  two  centigrams  of  cocain  solution  (i  per  cent.)  or  5-20 
c.c.  of  saline  solution  to  which  one  or  two  centigrams  of  cocain  are  added. 
The  needle  is  inserted  at  a  depth  of  3  or  4  cm.  This  method  while  less 
efficacious  than  the  preceding  one  as  far  as  duration  of  relief  of  pain  is 
concerned,  is  nevertheless  useful.  It  can  be  applied  not  only  in  sciatica, 
but  also  in  gastric  and  vesical  crises  of  Tabes  (Laven,  Centralbatt  f.  Chir., 
1910),  in  lead  colic  (Achard,  Gaz.  hebd.  de  med.  et  chir.,  1901),  in  cancer  of 
the  vertebrae  (Chipault,  Congres  de  Chir.,  1901).  Langbein  (Deut.  Mediz. 
Wchn.,  1913)  uses  1  grm.  of  novocain  mixed  with  one  quarter  its  weight 
of  sodium  bicarbonate  and  one-half  its  weight  of  sodium  chlorid.  The 
powder  is  dissolved  in  100  c.c.  of  distilled  water,  cold,  and  the  mixture  is 
injected.  After  the  injection  the  patient  reclines  with  the  shoulders 
raised  and  the  legs  low.  If  there  is  a  tendency  to  weakness,  the  patient 
is  laid  horizontal  for  a  few  minutes.  In  fifteen  to  twenty  minutes  all 
symptoms  of  sciatica  disappear,  but  he  keeps  the  patient  in  bed  for  two 
days.  Out  of  twelve  patients  seven  were  permanently  cured.  For 
details  of  epidural  injections  consult  Sicard  and  Cathelin  (Compt.  rend. 
Soc.  de  Biol.,  1910). 

Rosi  {Policlinico,  1912)  has  applied  in  eleven  cases  Baccelli's  method 
of  intravenous  injection  of  a  very  weak  solution  of  carbolic  acid  in 
treatment  of  chronic  sciatica  (1  per  cent,  or  1  per  thousand  solution). 
He  obtained  complete  recoveries;  from  ten  to  sixty  injections  were 
required  for  the  purpose. 

In  grave  and  protracted  cases,  when  the  above  treatment  has  failed, 
surgical  procedures  must  be  resorted  to.  Nerve-stretching,  excision  of 
portions  of  the  nerve  sometimes  give  relief.  For  more  details  see  Treat- 
ment of  Neuralgia  in  general. 

Recently  Pers  (Ugeskrift  fur  Laeger,  Copenhagen,  October,  1911) 
devised  a  method  called  "neurolysis,"  which  he  applied  to  seventy  cases. 
After  a  considerable  interval  forty-seven  out  of  fifty-eight  were  found 
permanently  cured  as  also  two  others  after  a  second  neurolysis.  The 
method  consists  of  freeing  the  sciatic  nerve  from  all  adhesions  that  may 


444  NEURALGIA 

have  developed  as  the  result  of  perineuritis  and  which  according  to  the 
author  are  responsible  for  persistent  sciatica.  The  incision  along  the  back 
of  the  femur,  from  the  lower  margin  of  the  gluteus  maximus  down  to  and 
below  the  middle  of  the  thigh.  The  nerve  is  sought  between  the  vastus 
externus  and  biceps  and  with  the  finger  detached  from  its  adhesions,  work- 
ing always  from  below  upward  to  the  sciatic  foramen.  Baracz  modified 
this  method:  he  works  his  finger  through  the  gluteus  maximus  fibers 
down  to  the  nerve  where  it  enters  the  notch  and  breaks  the  adhesions. 

The  methods  of  Pers  and  Baracz  may  be  successful  in  uncomplicated 
typical  sciatica  without  two  much  atrophy.  If  the  sciatica  is  associated 
with  pain  in  adjoining  regions,  especially  along  the  crural  nerve  and  up- 
ward in  the  lumbar  region,  there  is  evidently  an  involvement  of  the 
plexus  and  no  relief  can  be  expected  from  neurolysis.  To  detect  an  old 
fracture  or  old  arthritis  of  the  lowest  lumbar  vertebrae  or  of  sacro-iliac 
articulation,  which  all  may  simulate  sciatica,  an  examination  with 
Roentgen  rays  is  necessary  before  neurolysis  is  decided  upon. 

F.  Lumbar  Neuralgia. — This  form  concerns  the  branches  of  the 
lumbar  plexus. 

Etiology. — Cold,  rheumatism,  grippe,  malaria,  diabetes,  anaemia  are 
the  general  causes.  Locally  the  nerves  may  be  compressed  by  pelvic, 
renal  and  mesenteric  tumors. 

Symptoms. — According  to  the  branches  of  the  plexus  involved  the 
neuralgis  will  be  present  in  different  areas. 

(a)  In  Lurnbo-abdominal  neuralgia  the  pain  is  in  the  lumbar  region, 
lower  abdominal  area,  scrotum  and  spermatic  cord.  To  this  category 
belongs  also  neuralgia  of  the  testicle.  During  the  paroxysmal  attacks 
the  abdominal  muscles  may  become  contracted.  The  skin  may  be  hy- 
peraesthetic,  but  in  old  cases  it  is  usually  hypaesthetic.  Trophic  and 
vasomotor  disturbances  (herpes,  etc.)  may  be  observed.  Spontaneous 
ejauculation  may  occur. 

(b)  Obturator  neuralgia  is  associated  with  an  obturator  hernia.  The 
pain  radiates  to  the  internal  surface  of  the  thigh. 

(c)  In  Crural  neuralgia  the  pain  extends  along  the  course  of  the  crural 
nerve  to  the  inner  surface  of  the  leg  and  foot.  The  tender  spots  are  found 
in  the  inguinal  region,  on  the  inner  surface  of  the  knee,  internal  malleolus 
and  inner  border  of  the  foot. 

(d)  Neuralgia  of  the  femoro-cutaneous  nerve  is  known  under  the  name 
of  Meralgia  paraesthetica.  It  was  first  described  by  Roth  and  Bernhardt 
in  1895.  As  the  name  implies,  there  is  not  only  a  neuralgic  pain,  but  also 
paraesthesia.  The  latter  consists  of  a  burning  sensation  and  a  numbness 
over  the  antero-external  surface  of  the  thigh.     The  burning  sensation  is 


NEURALGIA  445 

slightly  decreased  by  flexing  the  thigh  on  the  pelvis  and  the  leg  on  the  thigh. 

The  paresthetic  disturbances  are  continuous  irrespective  of  the  position 
of  the  body,  but  the  walking  or  any  displacement  of  the  limb  increases 
their  severity  and  sometimes  causes  intolerable  pain.  Generally  rest  gives 
relief,  but  in  some  cases  immobility  increases  the  suffering. 

The  tactile  sense  is  diminished  and  the  patient  feels  at  each  contact 
that  something  is  interposed  between  the  thigh  and  the  hand  applied  to  it. 
In  some  cases  the  least  touch  provokes  severe  pain.  The  diminished  tactile 
sense  (hypaesthesia)  is  frequently  associated  with  a  diminished  pain  sense 
(hypalgesia)    and  thermic  sense. 

The  tender  spots  characteristic  of  neuralgia  in  general  are  found  here 
at  the  point  of  emergency  of  the  external  cutaneous  nerve  from  the  crest 
of  the  ilium. 

The  causes  of  meralgia  paraesthetica  are:  trauma,  cold,  infectious  dis- 
eases, intoxications,  constitutional  diseases.  The  most  frequent  cause  is 
injury.  The  anatomical  relations  of  the  external  cutaneous  nerve  make 
it  vulnerable:  the  nerve  is  placed  in  a  muscle  indispensable  to  standing 
and  walking  (psoas)  and  in  a  muscle  (fascia  lata)  the  contraction  of  which 
in  walking  presses  upon  it  and  stretches  it. 

The  anatomical  examination  made  in  a  few  cases  has  shown  that  the 
affection  is  due  to  a  neuritis. 

Treatment— A  prolonged  rest  may  in  some  cases  give  relief.  Sulphur 
baths,  massage,  galvanism,  internal  administration  of  iodides  have  been 
advised.  In  obstinate  cases  resection  of  the  nerve  is  the  only  means  to  be 
used. 

Meralgia  paraesthetica  is  not  infrequently  associated  with  inter- 
mittent claudication."  This  phenomenon,  to  which  Charcot  first  called 
attention  in  1856,  is  characterized  by  intense  pain  in  the  calves  of  the  legs 
and  difficulty  of  walking  appearing  a  few  minutes  after  the  patient  begins 
to  walk.  As  soon  as  he  sits  down,  the  pain  begins  to  subside  and  finally 
disappears.  According  to  Charcot,  the  paroxysms  are  due  to  contraction 
and  obliteration  of  the  arteries  in  the  affected  limbs. 

Men  are  much  more  frequently  affected  than  women.  Russians  and 
especially  Hebrews  are  particularly  predisposed  to  this  affection.  Erb 
made  a  statistical  study  of  the  etiological  factors  {Munch.  Med.  Wchn., 
1910)  and  he  reaches  the  following  conclusions:  Syphilis  which  is  so  fre- 
quently the  cause  of  arteritis,  plays  apparently  a  slight  role  in  intermittent 
claudication.  He  found  only  8  per  cent,  cases  of  syphilis.  Gout,  diabetes, 
alcohol  play  a  very  negligeable  part.  Tobacco,  however,  is  apparently 
a  prominent  etiological  factor.  Over  a  half  of  Erb's  cases  were  heavy 
smokers. 


44^  NEURALGIA 

Besides  these  general  causes,  local  causes  have  an  important  place. 
Cold  is  particularly  frequent. 

As  to  the  clinical  forms  of  intermittent  claudications  the  following  are 
observed: 

(i)  Mild  form,  in  which  the  entire  condition  consists  of  a  sensation 
of  weakness  and  fatigue,  slight  pain,  some  paraesthesia.  They  all  disappear 
when  the  patient  walks  slowly. 

(2)  Severe  form  in  which  all  the  symptoms  are  very  pronounced  from 
the  beginning. 

(3)  Association  with  Meralgia  paraesthetica  (see  above). 

(4)  Vaso-motor  form,  in  which  the  vaso-motor  disturbances  predomi- 
nate over  the  other  symptoms.  Here  the  individual  complains  of  "dead 
feet."  The  latter  appear  either  pale  or  red.  Goldflam  {Neur.  Centr.,  1910) 
described  the  following  phenomenon.  Normally  when  the  leg  is  extended 
several  times  in  succession,  and  then  left  to  drop,  the  foot  becomes  very 
pale;  the  pallor  gradually  disappears  to  be  replaced  by  a  hyperaemia.  In 
cases  of  intermittent  claudication  this  phenomenon  is  produced  with  great 
facility  and  is  very  pronounced. 

The  diagnosis  depends  considerably  upon  the  state  of  the  pulse  in  the 
pedis  dorsalis  artery  and  posterior  tibial  artery.  The  pulse  may  be  very 
small  or  totally  absent.  For  differential  diagnosis  with  spinal  intermittent 
claudication  see  page  334. 

The  prognosis  must  be  made  guardedly,  as  the  condition  may  precede 
an  eventual  gangrene  through  the  endarteritis.  However  great  ameliora- 
tion may  be  expected  from  proper  management. 

Treatment. — An  effort  should  be  made  to  remove  the  cause.  The 
general  and  local  circulation  must  be  improved.  Rest  in  a  horizontal 
position,  local  heat  and  perhaps  galvanism — may  all  be  of  benefit. 

In  meralgia  paraesthetica  the  intermittent  lameness  is  clinically  the 
exact  reproduction  of  Charcot's  type,  but  the  etiological  factor  is  different. 

G.  Rare  Forms  of  Neuralgia. 

(a)  Coccygodynia  or  Coccygeal  Neuralgia. — It  occurs  mostly  in  women. 
Trauma  is  a  frequent  cause.  Cold,  protracted  labor  may  also  produce  it. 
Hysteria  and  neurasthenia  are  the  predisposing  causes.  The  disease  is 
characterized  by  a  pain  in  the  region  of  the  coccyx.  The  pain  is  increased 
in  walking,  defecation,  micturition.  The  normal  sitting  position  is  ex- 
tremely painful.  Rectal  examination  shows  extreme  tenderness  of  the 
coccyx. 

The  treatment  consists  of  faradization  of  the  coccyx  and  rectal  supposi- 
tories of  opium  or  cocain.  When  hysteria  or  neurasthenia  are  the  under- 
lying causes,   an  appropriate  treatment  of  these  affections  is  the  first 


HERPES    ZOSTER  447 

indication.     The  disease  is  generally  very  obstinate.     Extirpation  of  the 
coccyx  is  sometimes  the  only  remedy. 

(b)  Spermatic  neuralgia  is  characterized  by  pain  along  the  spermatic 
cord  extending  to  the  scrotum  and  epididymis. 

(c)  Perineal  neuralgia  is  characterized  by  pain  radiating  from  the 
perineum  to  the  penis,  producing  a  desire  for  micturition  which  then  be- 
comes painful. 

(d)  Vesical,  rectal,  urethral  neuralgias  may  also  occur. 

(e)  Metatarsalgia  (Morton's  disease),  which  is  characterized  by  pain 
in  the  fourth  metatarsophalangeal  articulation,  sometimes  also  in  the 
second,  is  considered  by  some  as  the  result  of  articular  or  bony  changes  and 
by  others  as  due  to  a  neuralgia  of  the  external  plantar  nerve.  Tightly 
fitting  shoes  or  prolonged  standing  are  probably  the  causes  of  this  affection. 
The  treatment  consists  of  avoiding  pressure  on  the  heads  of  the  metatarsal 
bones.  Wide  shoes  may  help.  In  protracted  cases  removal  of  the  head  of 
the  affected  metatarsal  is  the  only  means. 

HERPES  ZOSTER  (ZONA) 

This  affection  should  be  considered  here,  as  it  is  very  frequently  asso- 
ciated with  neuralgia  (see  preceding  chapter). 

It  is  characterized  by  an  acute  vesicular  eruption  developed  upon  an 
erythematous  base  and  following  the  course  of  a  nerve  or  several  nerves; 
it  is  accompanied  by  neuralgic  pain. 

Pathology. — The  lesion  of  herpes  zoster  is  not  definitely  settled.  Some 
believe  that  it  is  due  to  a  peripheral  neuritis.  Others  bring  forward  ample 
proofs  of  an  involvement  of  the  spinal  ganglia  (acute  inflammation  or 
hemorrhage)  or  of  the  Gasserian  ganglion  in  cases  of  zona  following  the 
course  of  the  fifth  nerve.  When  the  spinal  ganglia  are  at  fault,  a  secondary 
degeneration  develops  in  the  posterior  roots  and  hence  in  the  peripheral 
nerves.  Some  observers  believe  that  the  primary  lesion  lies  in  the  spinal 
cord.  The  researches  of  Hedinger  (Deut.  Ztschr.  f.  Nerv.,  1903),  of  Dejeine 
and  Thomas  {Revue  Neur.,  1907),  of  Head  and  Campbell  (Brain,  1909) 
show  that  the  ganglia  and  both  portions  (peripheral  and  central)  of  the 
roots  are  chiefly  involved.  Laminiere  (Rev.  Neur.,  1907)  has  shown  also 
a  degeneration  of  communicating  branches  of  the  sympathetic  which 
corresponded  to  changes  in  the  lateral  columns  of  cells  in  the  gray  matter. 
A  radiculo-ganglionic  lesion  accompanied  sometimes  by  a  lesion  in  the 
cord  is  the  anatomical  basis  of  herpes  zoster. 

Head's  explanation  of  the  pain  and  eruption  is  very  interesting.  A 
lesion  of  a  spinal  ganglion  will  produce  a  continuous  sensory  impression 


448  HERPES    ZOSTER 

which  is  directed  towards  the  centers,  hence  the  pain.  On  the  other 
hand,  a  peripheral  sensory  fiber  containing  vasomoter  fibrils  of  a  centrif- 
ugal nature  will  transmit  to  the  cutaneous  blood-vessels  the  irritation 
caused  by  the  lesion  in  the  ganglion;  hence,  the  erythema  upon  which 
the  herpetic  eruption  appears. 

As  to  the  pathogenesis  of  zona,  within  the  last  few  years  micro- 
organisms have  been  observed  by  some  authors  in  cultures  obtained 
from  the  cerebro-spinal  fluid  of  patients  suffering  from  herpes  zoster. 
Very  recently  (Deut.  Mediz.  Wchn.  N°  18,  1913)  Sunde  reported  a  case 
of  ophthalmic  zona  which  came  to  autopsy,  and  a  coccus  was  found  in 
large  quantities  in  the  Gasserian  ganglion.  The  micro-organism  was  es- 
pecially in  abundance  in  the  hemorrhagic  foci  and  small  blood-vessels. 
The  ganglion  was  in  a  state  of  acute  inflammation;  small  hemorrhages 
and  round-cell  infiltration  were  marked. 

Symptoms. — Pain  and  eruption  are  the  characteristic  signs  of  the 
affection.  Pain  is  shooting  in  character.  According  to  the  localization 
of  the  zona  special  symptoms  will  be  present. 

(a)  Intercostal  Zona. — This  is  the  most  frequent  form.  The  onset 
of  the  disease  is  usually  preceded  by  fever  and  general  malaise.  The  de- 
velopment of  the  vesicles  may  be  sudden  or  gradual  but  commonly  it  ap- 
pears on  the  third  or  fourth  day.  Pain  may  precede  the  eruption  or  follow 
it.     Besides  pain  there  may  be  a  burning,  itching  or  tingling  sensation. 

The  eruption  may  follow  the  course  of  an  intercostal  nerve,  anteriorly 
and  posteriorly,  but  sometimes  it  has  a  different  direction  and  crosses  the 
nerves.  At  first  the  skin  is  only  erythematous,  but  soon  elevations  are 
noticeable  in  areas.  The  center  of  the  latter  becomes  a  vesicle;  the  con- 
tents of  the  vesicle  is  at  first  serous,  but  later  becomes  purulent  and  hemor- 
rhagic. The  lymphatic  glands  in  the  vicinity  become  enlarged  in  the 
majority  of  cases.  The  bleb  soon  dries;  on  the  fifth  or  tenth  day  a  crust 
is  formed  and  when  it  falls  off,  a  small  scar  is  left.  Usually  when  the 
rash  begins  to  heal,  the  pain  disappears,  but  in  elderly  individuals  it  per- 
sists months  after  the  eruption  has  ceased  to  exist. 

The  affected  area  presents  objective  sensory  disturbances:  there  may 
be  hyperesthesia  or  anaesthesia.  The  latter  is  present  especially  over 
deep  and  extensive  scars.    • 

The  affection  lasts  from  several  days  to  several  weeks. 

(b)  Ophthalmic  Zona. — There  is  usually  no  prodromal  period.  The 
symptoms  may  consist  only  of  an  erythema  with  cedema  of  the  eyelid. 
In  the  majority  of  cases  vesicles  appear  with  very  violent  neuralgic  pain  on 
the  forehead  near  the  middle  fine,  on  the  upper  eyelid,  and  the  base  of  the 
nose.     The  center  of  the  eruption  is  the  supraorbital  foramen.     Sometimes 


HERPES    ZOSTER 


449 


the  rash  extends  to  the  side  of  the  nose  down  to  the  alae.  Hutchinson 
pointed  out  the  seriousness  of  the  latter  occurrence:  he  observed  in  such 
cases  almost  invariably  ulceration  of  the  cornea. 

Grave  symptoms  frequently  accompany  ophthalmic  zona,  viz.  con- 
junctivitis, keratitis  (neuro-paralytic) ,  iritis,  suppuration  of  the  cornea 
and  of  the  entire  globe.  Optic  neuritis,  paralysis  of  the  third  and  sixth 
nerves  have  been  observed  in  some  cases. 

(c)  Zona,  of  the  face  may  affect  all  the  sensory  branches  of  the  fifth 
nerve.  It  may  involve  the  lips,  nose  and  cheeks.  The  tongue  is  also  occa- 
sionally involved.  R.  Hunt  has  shown  that  a  herpetic  eruption  over  the 
auricle  and  in  the  external  meatus  is  due  to  an  inflammation  of  the 
geniculate  ganglion  (geniculate  poliomyelitis) .  It  is  frequently  associated 
with  palsy  of  the  seventh  nerve  (see  chapter  of  peripheral  facial  palsy). 

(d)  Zona,  of  the  extremities  may  follow  the  exact  course  of  individual 
nerves  or,  similarly  to  intercostal  herpes,  present  an  independent  or  irregu- 
lar distribution.  In  a  case  of  cervico-brachial  herpes  zoster  observed 
by  me  {Amer.  Medicine,  1909)  on  an  aged  woman  excruciating  pain  was 
followed  three  days  later  by  a  vesicular  eruption  extending  from  above 
the  left  scapula  down  over  the  external  aspect  of  the  left  upper  limb  to 
the  wrist. 

(e)  Zona  may  occur  on  the  abdomen,  dorsum,  neck  and  genitalia. 
Course,  Prognosis. — Apart  from  the  complications  of  the  ophthalmic 

form,  the  prognosis  is  usually  favorable.  Recurrences  take  place,  although 
rare.  In  one  of  my  patients,  a  man  of  twenty-nine,  three  attacks  occurred 
within  two  years  and  invariably  in  the  same  area  (intercostal).  Bilateral 
herpes  is  also  rare. 

Etiology. — Trauma  and  cold,  intoxications  with  carbonic  acid  gas,  lead 
and  arsenic  are  not  infrequently  traced  as  causes. 

It  has  also  been  observed  in  the  course  of  infectious  diseases,  (grippe, 
pneumonia,  etc.)  of  diabetes.  Finally  zona  may  develop  independently 
as  a  primary  affection  with  a  febrile  onset  and  glandular  swelling;  other- 
wise speaking,  as  an  infectious  disease.  The  analogy  with  the  onset  of 
acute  anterior  poliomyelitis  is  striking.  Epidemics  of  zona  have  been 
observed  by  a  number  of  writers.  Herpes  may  occur  at  any  age,  but  pre- 
dominately under  the  age  of  twenty-five.  Infants  are  also  subject  to  it. 
Aged  individuals  are  peculiarly  susceptible  to  it. 

Treatment. — The  primary  form  should  be  treated  as  any  other  infec- 
tious disease  by  diuretics,  purgatives  and  rest  in  bed.  The  secondary 
or  symptomatic  form  will  be  managed  according  to  the  original 
malady.  Locally  the  vesicles  should  never  be  pierced.  Applications  of 
sedative  ointments  or  liniments  with  opium  and  cocain  and  later  during 
29 


45°  HERPES    ZOSTER 

the  drying  stage  of  zinc  powder  are  usually  sufficient.  I  obtained  good 
results  with  application  of  ichthyol.  For  relief  of  pain  bromides  and 
coal-tar  products  can  be  given.  In  protracted  cases  arsenic  and  galvanism 
are  advisable.  Resection  of  the  posterior  spinal  roots  at  the  level  cor- 
responding to  the  area  involved  has.been  recommended  and  good  results 
reported  {Leriche.  Lyon  Chir.,  19 12). 


CHAPTER  XXV 

FUNCTIONAL  NERVOUS  DISEASES 

NEURASTHENIA  (NERVOUS  EXHAUSTION) 

Under  this  name  is  known  a  symptom-group  the  chief  characteristic 
of  which  is  a  persistent  neuro -muscular  fatigue  with  general  irritability. 

Beard  (in  1880)  was  the  first  who  grouped  together  the  individual 
symptoms  of  the  disease  which  prior  to  him  were  attributed  simply  to 
nervousness. 

Symptoms. — (a)  Diminution  of  Muscular  Energy  is  the  most  promi- 
nent symptom.  It  is  manifested  in  a  lassitude  which  interferes  with  the 
patient's  daily  work.  The  usual  physical  exercises  cannot  be  carried  out. 
The  least  exertion  brings  on  an  undue  fatigue.  In  some  cases  the  tired 
feeling  is  felt  only  in  the  legs,  in  others  in  the  entire  body.  In  extreme 
cases  the  patient  refuses  to  stand,  walk  or  move  from  one  place  to  an- 
other. The  characteristic  feature  of  this  fatigue  is  that  it  is  particularly 
marked  in  the  morning.  In  such  cases  the  sleep  is  not  refreshing  to  the 
patient;  in  getting  up  he  feels  as  if  he  had  not  spent  the  night  in  bed. 

In  other  cases  the  fatigue  is  not  continuous,  but  paroxysmal.  The 
patient  gets  some  relief  for  a  few  hours  and  then  suddenly  upon  a  slight 
exertion  or  even  without  it  begins  to  feel  the  fatigue. 

(b)  Neurasthenics  very  frequently  complain  of  backache.  In  some 
cases  it  is  not  a  genuine  pain,  but  a  pressure,  a  burning  or  only  an  inde- 
scribable discomfort  along  the  spine.  These  sensations  usually  increase 
upon  exertion  or  more  or  less  prolonged  standing.  The  skin  over  the  spine 
is  over-sensitive,  so  that  the  least  touch  or  even  the  contact  of  the  clothes 
makes  the  patient  uncomfortable.  The  most  frequent  seat  of  the  back- 
ache is  the  lower  (sacral)  portion  of  the  spinal  column. 

(c)  Headache  is  a  common  occurrence.  It  consists  frequently  of  a 
sense  of  fullness,  heaviness,  pressure  or  of  a  band-like  constriction  around 
the  head  (neurasthenic  lead-cap).  It  is  sometimes  confined  to  the  fore- 
head, the  temples,  but  most  frequently  to  the  occipital  region.  In  some 
cases  instead  of  fullness  there  is  a  sensation  of  emptiness,  of  a  vacuum. 
In  other  cases  the  headache  is  accompanied  by  vertigo,  noises  in  the  ears, 
dimness  of  vision. 

The  headache  may  be  continuous  or  paroxysmal.  It  is  particularly 
evident  in  the  morning. 

45i 


452  NEURASTHENIA 

(d)  Gastro -intestinal  Disorder  is  a  very  frequent  symptom.  In  the 
mild  form  there  is  usually  preservation  of  appetite.  The  patient  enjoys 
his  food,  but  an  hour  after  the  meals  a  discomfort  appears.  Fullness  and 
pressure  in  the  epigastrium,  eructations,  are  tormenting  the  patient.  At 
the  same  time  he  feels  oppressed,  flushes  of  heat  go  to  his  head,  the  heart 
palpitates.  He  feels  somnolent,  heavy.  This  condition  lasts  during  the 
entire  process  of  digestion. 

Constipation  is  a  usual  accompaniment  of  neurasthenic  dyspepsia. 

As  to  the  chemical  processes  of  digestion,  the  latest  observations 
show  that  they  are  about  normal  in  mild  cases.  The  dyspeptic  symp- 
toms are  due  to  a  deficient  motor  innervation  of  the  gastro-intestinal 
tract  fatonyj.  It  is  also  interesting  to  notice  that  in  spite  of  the  digestive 
disorder  the  patients  do  not  lose  in  weight. 

When,  however,  the  digestion  is  considerably  disturbed,  the  general 
nutrition  suffers.  In  such  cases  there  is  a  marked  diminution  of  free 
hydrochloric  acid  and  the  constipation  is  obstinate.  The  patient  loses  in 
weight,  he  is  emaciated  and  pale. 

(e)  Insomnia,  while  not  constant,  is  quite  frequently  met  with.  Either 
the  patient  has  great  difficulty  to  fall  asleep  or  awakes  several  times 
during  the  night  in  a  state  of  anxiety  and  excitement.  As  a  rule  the 
neurasthenic  sleep  is  incomplete.  Terrifying  dreams  are  very  frequently 
observed. 

(/)  Mental  Fatigue  almost  always  accompanies  muscular  fatigue.  The 
majority  of  the  neurasthenics  suffer  from  inability  to  concentrate  their 
thoughts  upon  one  subject  for  any  reasonable  length  of  time.  They  are 
absent-minded,  incapable  to  solve  problems  of  a  more  or  less  complex 
nature.  Figuring  becomes  a  difficult  task.  A  long  conversation  on  a 
serious  subject  is  impossible.  They  are  hesitating  in  their  actions  and 
decisions.  The  least  attempt  to  resume  their  usual  mental  occupation 
brings  on  headache. 

In  some  cases,  however,  the  patient  retains  his  mental  energy,  but 
the  muscular  fatigue  is  persistent.  In  others  the  mental  fatigue  appears 
only  after  a  certain  number  of  hours'  work.  There  are  great  variations 
in  the  degree  and  intensity  of  the  mental  exhaustion. 

The  disposition  of  the  patient  usually  undergoes  a  change.  He  is 
depressed,  discouraged,  highly  irritable,  cannot  stand  contradiction. 
He  avoids  his  best  friends,  wants  to  be  let  alone.  He  is  pessimistic  and 
does  not  find  pleasure  in  anything. 

(g)  Circulatory  disturbances  are  very  frequent.  Cardiac  palpitation 
occurs  upon  the  least  emotion.  Sensations  of  cold  or  heat  along  the  spine 
or  in  the  extremities,  lowering  of  vascular  tension,  are  all  due  to  deficient 


NEURASTHENIA  453 

tone  of  the  vasomotor  apparatus.  The  patient's  skin  and  especially  the 
hands  and  feet  are  moist,  clammy. 

The  six  symptoms  just  described  are  characteristic  of  neurasthenia. 
There  are  some  minor  manifestations  which  are  not  observed  in  every 
case,  but  frequent  enough  to  deserve  mentioning. 

Sensory  disturbances  may  be:  hyperesthesia  generalized  or,  more 
frequently,  localized;  various  paresthesias,  as  tingling,  numbness,  burn- 
ing, itching  or  pain.  Pruritus  ani,  prurigo  in  general  may  occur  in  neuras- 
thenics. Neurasthenia  predisposes  the  individual  to  neuralgia.  The 
special  senses  may  also  be  disturbed.  The  eyes  get  easily  fatigued.  As 
soon  as  the  patient  begins  to  read,  the  letters  become  blurred  and  the  eye- 
globes  painful.  The  hearing  is  somewhat  affected;  the  least  noise  gives 
a  painful  sensation  in  the  ears;  various  noises  are  heard  by  the  patient 
The  taste  and  smell  also  suffer  sometimes. 

Motor  disturbances  are  sometimes  manifested  in  cramps  in  the  legs 
and  tremor.     The  latter  is  seen  in  the  tongue  and  fingers. 

Vertigo  is  occasionally  observed.  It  may  be  continuous  or  paroxysmal. 
In  the  latter  case  it  occurs  mostly  in  the  morning. 

Secretory  disturbances  are  various.  Either  there  is  a  diminution  or 
increase  of  secretion.  In  the  first  case  the  skin  and  the  m.ucous  membranes 
are  dry;  the  quantity  of  urine  is  diminished.  In  the  second  case  the 
perspiration  and  the  amount  of  urine  are  abundant.  Polyuria  and 
phosphaturia  are  sometimes  observed. 

Sexual  Disturbances  are  as  a  rule  present  In  the  average  case  it 
consists  of  some  degree  of  impotence.  In  some  cases,  however,  the  disor- 
der in  the  function  of  the  genitalia  is  so  predominant  that  the  disease 
deserves  the  special  name:  "sexual  neurasthenia."  Sexual  excitement, 
priapism,  nocturnal  emissions,  premature  ejaculation,  spermatorrhea, 
burning  sensation  in  the  urethra,  extreme  tenderness  of  the  scrotum, 
testicles,  penis,  are  all  symptoms  of  this  form  of  neurasthenia.  Added 
to  the  other  symptoms  of  the  disease,  they  put  the  patient  in  a  state  of 
extreme  anxiety.  Gradually  his  sexual  desire  decreases  and  even  disap- 
pears. The  latter  together  with  impotence  and  frequent  emissions  has  an 
unusual  depressing  effect  on  the  sufferer. 

Course,  Prognosis. — When  the  cause  is  removed  and  the  proper 
treatment  promptly  instituted,  recovery  may  follow  in  a  few  weeks. 
The  duration  of  the  malady  depends  upon  these  two  factors.  It  should, 
however,  be  borne  in  mind  that  recurrences  are  not  infrequent.  Generally 
speaking,  the  prognosis  is  favorable.  Except  the  cases  in  which  the 
neurasthenic  symptoms  are  secondary  to  some  grave  disease  (tuberculosis, 
syphilis,  etc.),  neurasthenia  is  a  curable  disease. 


454  NEURASTHENIA 

Pathogenesis. — There  are  no  absolutely  certain  facts  concerning  the 
nature  of  neurasthenia.  Various  theories  have  been  advanced.  Whether 
in  the  course  of  neurasthenia  some  toxic  material  circulates  in  the  blood 
and  produces  the  fatigue  in  the  nervous  and  muscular  systems,  or  else  the 
nervous  system  is  affected  first  from  overuse  and  the  other  organs  are 
disturbed  in  their  nutrition  secondarily,  it  is  impossible  to  tell  in  the 
state  of  our  present  knowledge.  The  cells  of  various  cerebral  centers 
primarily  and  systems  of  neurones  secondarily  are  probably  disturbed  in 
their  function. 

Diagnosis. — Continuous  fatigue  in  the  physical,  intellectual  and 
moral  spheres,  especially  manifested  upon  the  least  physical  or  mental 
exertion,  irritability,  prolonged  backache  and  headache,  insomnia,  gastro- 
intestinal atony,  are  all  symptoms  sufficiently  characteristic  of  neuras- 
thenia. Each  of  these  symptoms  individually  taken  may  be  observed  in 
otherMiseases.  It  is  therefore  essential  that  the  entire  symptom-group 
be  present. 

There  are  some  organic  nervous  or  mental  diseases  which  may  be 
preceded  by  a  prolonged  period  of  symptoms  resembling  neurasthenic 
manifestations.  The  prodromal  stage  of  paresis,  of  melancholia  and 
mania  are  characterized  by  such  symptoms.  They  resemble,  however, 
neurasthenia  only  superficially,  they  never  present  the  true  and  complete 
picture  of  the  latter.  They  are  merely neurasthenoid  (neurasthenia-like). 
The  differentiation  is  therefore  necessary,  as  the  prognosis  will  be  radically 
different. 

Neurasthenic  symptoms  may  be  observed  in  the  course  of  cerebral 
tumors,  of  tabes,  of  cerebral  syphilis,  of  exophthalmic  goiter.  A  careful 
examination  is  necessary  in  every  case  with  a  neurasthenic  symptom- 
group,  as  an  organic  disease  of  the  nervous  system  may  be  overlooked. 
This  occurrence  is  particularly  noticeable  during  the  earliest  period  of 
paresis  (see  page  436). 

Neurasthenia  is  frequently  associated  with  hysteria.  As  the  symp- 
toms of  the  former  are  mostly  subjective  and  of  the  latter  objective,  there 
will  be  no  great  difficulty  in  differentiating  them. 

Etiology. — Neurasthenia  is  a  very  common  affection.  Excesses  of  any 
kind,  sexual,  alcoholic  and  others,  masturbation,  prolonged  and  uninter- 
rupted intellectual  effort,  depressive  emotions,  fright,  anxiety,  worry, 
traumatism,  are  the  most  frequent  causes  of  nervous  exhaustion.  Neuras- 
thenia is  also  observed  in  syphilis;  it  follows  an  attack  of  grippe,  typhoid 
fever  or  any  other  protracted  infectious  disease.  The  symptom-group 
accompanies  also  tuberculosis,  anaemia,  chlorosis,  lead  intoxication.  This 
is  the  so-called  "Neurasthenia  symptomatica." 


NEURASTHENIA  455 

A  neurasthenic  state  develops  more  easily  in  individuals  with  a  path- 
ologic heredity  (nervous  or  mental  diseases),  as  their  predisposed  nervous 
system  is  more  apt  to  succumb  under  the  influences  mentioned  above 
than  in  persons  with  a  normal  make-up.  It  should  not  be  forgotten  that 
nervous  exhaustion  may  occur  in  any  individual  subject  to  the  effect  of 
the  described  causative  factors.  The  latter  may  make  a  perfect  recovery, 
while  the  former  is  easily  apt  to  suffer  recurrences. 

Treatment. — The  first  indication  in  neurasthenia  is  to  combat  its 
most  distressing  symptom,  viz.  fatigue.  Physical  and  mental  rest  is 
the  most  important  element  of  the  treatment.  When  the  tired  feeling  is 
marked,  the  patient  must  be  put  to  bed.  While  in  bed  all  mental  exertion 
should  be  avoided.  He  should  be  kept  away  from  all  possible  causes  of 
worriment  or  excitement.  The  latter  can  be  accomplished  when  the 
patient  is  isolated  and  placed  under  the  immediate  care  of  an  intelligent 
nurse.  Full  and  nutritious  feeding  is  necessary.  The  state  of  gastro- 
intestinal digestion  should  be  taken  into  consideration  in  each  individual 
case.  Atony  of  the  bowels  can  be  combated  by  massage  of  the  abdo- 
men, by  internal  administration  of  laxatives,  among  which  cascara 
sagrada  is  the  most  desirable,  by  daily  ingestion  of  sufficient  amount  of 
fruit  or  green  vegetables.  Atony  of  the  stomach  can  also  be  remedied 
by  local  massage  over  the  gastric  region  and  by  administration  of  bitters, 
as  mix  vomica  with  gentian,  nitro-muriatic  acid,  etc.,  immediately  before 
meals.  The  feeding  should  be  done  regularly  three  or  four  times  a  day. 
Stimulants  of  all  kinds  are  forbidden.  Coffee  or  tea  may  be  given  in 
very  small  amount  and  forbidden,  if  the  sleep  is  disturbed.  In  selecting  a 
diet,  special  attention  must  be  given  to  the  character  of  food.  Some 
patients,  for  example,  refuse  to  drink  large  quantities  of  milk.  It  is 
advisable  in  such  cases  to  have  the  patient  take  the  milk  at  first  in  very 
small  quantities  and  then  very  gradually  increase  the  amount  of  it.  By 
doing  so  I  have  succeeded  in  a  number  of  instances  in  making  the  most 
stubborn  patients  drink  large  quantities  of  milk.  Milk  is  an  ideal  food 
for  patients  confined  to  bed.  If  in  exceptional  cases  it  causes  gastric 
disturbances  in  spite  of  all  possible  precautions,  it  should  be  discontinued. 
Vegetables,  fruit,  small  amount  of  meat,  eggs,  custards,  are  very  nutritious 
articles  for  neurasthenics.  Sweets  and  very  starchy  food  should  be  avoided. 
Drugs  are  not  absolutely  necessary  in  every  case.  Glycero-phosphates, 
lecithin  are  excellent  in  asthenic  conditions  of  the  nervous  system. 
Iron  and  arsenic  are  indicated  if  the  blood  examination  shows  a  state  of 
anaemia.  In  case  of  insomnia  or  only  disturbed  sleep  sedatives  should  be 
avoided  as  long  as  possible.  It  can  be  combated  sometimes  by  simple 
measures,  as  a  cool  sponge  bath] or  a  tepid  general  bath  of  ten  minutes' 


456  PSYCH  ASTHENIA 

duration;  sometimes  also  by  the  application  of  a  cold  wet  towel  to  the 
neck.  If  these  means  fail,  bromides,  veronal,  sulfonal,  trional,  medinal^ 
may  be  tried.  Ten  grains  of  any  of  these  drugs  is  generally  an  average 
dose.     Of  course  the  latter  will  be  increased  according  to  the  indications. 

A  prolonged  rest  in  bed  is  not  without  some  inconveniences,  as  it  may 
lead  to  loss  of  appetite,  to  retardation  of  all  the  functions,  to  muscular 
wasting.  To  obviate  these  disturbances,  S.  Weir  Mitchell  recommended 
to  associate  with  the  rest  in  bed  also  massage,  passive  movements  and 
electricity.  At  first  these  measures  may  be  applied  daily,  but  later  only 
every  other  day.  By  these  means  the  general  nutrition  is  benefited 
considerably. 

Hydrotherapy  is  a  very  useful  adjuvant.  It  can  be  administered  as  a 
brief  douche,  a  sponging  or  ablution.  The  temperature  of  the  water  will 
vary  according  to  the  case.  Some  patients  cannot  stand  cold,  some  pre- 
fer tepid  water,  others  feel  better  after  hot  water.  A  gentle  general 
rubbing  after  the  application  of  water  is  not  to  be  neglected. 

In  mild  cases  of  neurasthenia  the  rest  may  not  necessarily  be  absolute. 
The  majority  of  the  cases  are  walking  neurasthenics,  who  are  compelled 
to  work.  In  such  cases  a  brief  relaxation  from  work  at  the  beginning  of 
the  treatment  should  be  insisted  upon.  If  it  is  impossible  to  obtain  it, 
the  patient  is  instructed  to  take  rest  as  much  and  as  often  as  he  can.  An 
hour's  relaxation  once  or  twice  a  day  will  be  very  refreshing  to  him.  Cool 
sponge-baths,  mornings  and  evenings,  followed  by  a  hard  rub,  will  help  the 
patient  considerably.  He  must  retire  early,  avoid  all  excitement,  in- 
cluding sexual  life;  otherwise  speaking,  he  must  secure  as  perfect  rest  as 
possible.  Smoking  must  be  done  very  moderately  and  avoided  at  night, 
as  it  is  an  excitant  to  some  individuals.     As  to  diet,  etc.,  see  above. 

NEURASTHENIC  PSYCHOSES  (PSYCHONEUROSES)  (PSYCHASTHENIA) 

Closely  associated  with  neurasthenia  are  certain  mental  disturbances 
which  develop  in  individuals  especially  predisposed,  viz.  neuropaths. 
The  latter  present  a  special  make-up  of  their  nervous  system  which  be- 
comes easily  affected  from  the  least  cause.  They  are  individuals  whose 
nervous  or  mental  equilibrium  has  not  a  solid  basis  and  is  constantly 
threatened  with  a  break.  They  are  usually  burdened  with  a  heavy  heredi- 
tary predisposition.  They  are  peculiar,  very  emotional,  impressionable, 
self-analyzing,  extremely  sensitive  and  scrupulous.  They  are  eccentric, 
dreamers,  with  romantic  tendencies.  They  are  subject  to  attacks  of 
great  anxiety,  to  morbid  fears,  obsessions.  The  mental  symptoms  may 
appear  at  various  periods  of  the  patient's  life,  but  they  ordinarily  develop 


PSYCHASTHENIA  457 

when  the  organism  is  in  a  state  of  exhaustion.  In  such  individuals  the 
neurasthenic  symptom-group  develops  with  the  greatest  facility  and  is 
characterized  by  its  special  tenacity  and  persistence. 

The  nerasthenic  manifestations  may  disappear,  while  the  psychic  dis- 
turbances will  persist.  Usually,  however,  they  develop  and  improve 
simultaneously. 

The  chief  characteristics  of  the  mental  symptoms  is  complete  lucidity 
of  the  patient's  mind,  his  complete  consciousness  of  their  presence.  The 
patient  realizes  the  absurdity  of  the  phenomena,  but  is  unable  to  overcome 
the  irresistible  sensations.  A  brief  description  of  the  psychic  disturb- 
ances is  necessary. 

A.  Phobia  or  Fear. — The  most  common  form  of  fears  is  agoraphobia. 
The  patient  fears  an  open  space.  He  will  avoid  crossing  a  large  avenue, 
a  boulevard  or  a  field. 

Monophobia  is  the  fear  to  be  alone. 

Claustrophobia  is  the  fear  of  a  closed  space.  The  number  of  phobias 
can  be  increased  indefinitely. 

To  this  category  belongs  also  a  special  phenomenon,  viz.  fear  of  touch- 
ing objects;  it  is  called:  Delire  du  toucher. 

A  fear  naturally  develops  a  state  of  anxiety.  If,  for  example,  the 
patient  suffers  from  agoraphobia  and  he  is  compelled  to  cross  a  field,  he 
will  become  agitated,  he  will  tremble,  he  will  make  several  attempts  to  do 
the  act,  his  heart  will  palpitate  violently,  he  will  perspire  abundantly. 
The  state  of  anxiety  will  be  so  great  that  he  will  have  to  abandon  his  plan, 
or  else  to  procure  himself  someone  who  will  accompany  him. 

B.  Doubts  or  Indecision.  Folie  de  Doute. — A  patient  affected  with 
morbid  doubts  may  reveal  the  state  of  his  mind  in  every  act  of  his  life. 
In  writing  a  letter,  for  example,  he  will  not  be  certain  whether  the  proper 
sentences  were  used  or  his  signature  is  placed.  He  will  therefore  tear  up 
the  envelope,  read  the  letter  over  again  or  write  another.  He  may  repeat 
the  same  act  several  times.  Another  patient  after  turning  out  the  gas 
in  his  room  at  night  may  be  tormented  by  the  possibility  of  a  mistake. 
He  will  get  up,  light  the  gas  again,  turn  it  out  and  a  few  minutes  later  per- 
form the  same  act  over  again.  Not  being  sure  of  the  correctness  of  their 
actions,  such  patients  are  continuously  in  a  state  of  doubt.  This  condition 
may  interfere  with  their  daily  occupations,  with  their  sleep  and  with  their 
whole  life.  They  feel  unhappy.  The  French  call  it  madness  of  doubt 
(Folie  de  doute.) 

C.  Obsessions, — Under  this  term  is  understood  a  state  of  mind  when 
a  certain  thought,  idea  or  image  invade  it  and  the  patient  is  unable  to 
free  himself  from  them.     He  realizes  the  groundlessness,  the  absurdity 


45^  HYPOCHONDRIA 

of  such  tenacious  ideas  or  images,  but  he  finds  himself  powerless  in  the 
presence  of  the  irresistible  force.  Kleptomania,  for  example,  is  a  variety 
of  obsessions.  The  patient  cannot  resist  the  irresistible  impulse  to  steal 
insignificant  objects.  One  of  my  female  patients  cannot  resist  a  knife. 
She  would  get  paroxysmal  desires  to  kill,  although  she  would  make  efforts 
to  avoid  the  sight  of  sharp  instruments.  Another  patient  was  obsessed  by 
the  idea  that  her  child  will  die  at  a  certain  hour.  In  some  cases  the  ob- 
sessions may  be  so  intense  that  irresistible  impulses  follow  and  crimes  are 
committed. 

What  characterizes  the  obsessions  is  the  complete  lucidity  of  mind. 
The  patient  is  perfectly  conscious  of  the  criminality  or  absurdity  of  a  cer- 
tain act.  He  struggles  against  the  thought  and  impulse,  he  suffers  morally. 
He  may  sometimes  overcome  the  fight,  but  sometimes  he  succumbs  and 
commits  a  criminal  act. 

D.  Abulia  or  Deficient  Will. — The  patient  thus  affected  has  not  the 
power  to  do  a  certain  act.  One  of  my  patients  while  walking  on  the 
street  could  not  step  over  a  loose  brick  or  a  leaf  of  a  tree.  Another 
patient  in  going  to  his  business  office  could  not  walk  over  the  shortest 
distance,  but  had  to  select  the  longest  route.  Should  these  patients, 
recognizing  the  ridiculous  side  of  their  condition,  attempt  to  overcome  it, 
they  would  be  thrown  into  a  state  of  anxiety,  would  tremble  and  even 
cry.     Their  suffering  is  indeed  great. 

E.  HYPOCHONDRIA 

This  affection  is  quite  frequent.  It  is  related  in  some  of  its  manifes- 
tations to  neurasthenia,  but  it  is  different  from  the  latter  in  its  essential 
features.  It  occurs  mostly  in  men  before  middle  life.  It  develops 
usually  upon  a  neuropathic  basis  (see  preceding  chapter) . 

Symptoms. — The  disease  is  characterized  by  a  vivid  sense  of  having 
one  or  more  organs  in  a  diseased  state.  Functional  changes  are  usually 
absent,  but  if  they  are  present,  they  are  extremely  slight.  The  patient 
complains  of  a  multitude  of  vague  symptoms  referable  to  one  or  several 
organs.  Believing  that  a  special  organ  is  affected,  he  -  will  observe  its 
function  from  day  to  day  or  from  hour  to  hour  and  the  most  insignificant 
symptom  will  be  interpreted  by  him  as  pathognomonic  of  some  disease 
in  a  state  of  development.  If  he  concentrates  his  thoughts  upon  the 
digestive  apparatus,  he  will  adopt  a  special  diet,  exclude  important 
articles  of  his  food,  change  the  regime  every  few  days  until  a  new  symptom 
from  another  source  will  make  its  appearance.  At  once  he  will  give  up 
the  idea  of  the  gastro-intestinal  tract  andattribute  his  illness  to  another 


HYPOCHONDRIA  459 

organ.  Should  he  notice  an  increase  or  diminution  in  the  daily  quantity 
of  urine  or  a  change  in  its  color,  his  thoughts  will  be  transferred  to  the 
kidneys.  One  of  my  patients,  a  male  of  thirty-eight,,  believed  two  years 
ago  that  his  testicles  were  in  a  diseased  state.  He  began  to  wear  a  sus- 
pensory padded  with  cotton  in  the  hottest  days  of  July,  would  apply 
extreme  heat  mornings  and  evenings,  so  that  the  scrotum  became  in- 
tensely erythematous.  Six  months  later,  subsequently  to  exposure,  he 
developed  a  mild  lumbago.  At  once  he  began  to  believe  that  the  origin 
of  his  troubles  lies  in  the  kidneys.  He  removed  the  suspensory  with  the 
cotton,  also  all  application  from  the  testicles  and  abandoned  himself  to 
the  analysis  of  the  renal  function.  His  urine  had  to  be  examined  chemi- 
cally and  microscopically  every  week.  Gradually  he  took  up  each  of 
the  abdominal  and  thoracic  viscera  and  now  he  is  under  the  impression 
that  his  brain  is  affected.  In  spite  of  his  continuous  preoccupation  over 
his  body,  he  never  missed  a  day  in  attending  to  his  affairs  (he  is  a  success- 
ful broker). 

In  another  series  of  cases  the  patients  complain  of  symptoms  referable 
to  the  entire  organism.  One  of  my  patients,  a  male  of  thirty-eight, 
has  disagreeable  sensations  in  the  region  of  the  liver  and  of  the  stomach, 
in  the  epigastrium;  he  has  palpitation  of  the  heart,  numbness  and  aching 
in  the  limbs,  shortness  of  breath,  pressure  and  pulling  sensations  about 
his  head.  A  thorough  examination  failed  to  reveal  physical  signs  of 
diseases  of  any  organ.  What  is  striking  about  these  patients  is  good 
general  development  of  the  body.  The  above  mentioned  patient  has  been 
suffering  for  several  years  and  still  he  has  a  healthy  appearance.  In 
some  cases,  however,  they  lose  in  weight,  especially  when  they  restrict 
their  diet  to  a  very  limited  amount  of  food  and  exclude  all  nutritious 
articles.  The  improper  food  selected  by  the  patient  himself  may  lead  to 
gastro-intestinal  disturbances. 

Believing  that  he  may  be  affected  with  some  pulmonary  disease, 
the  patient  will  take  special  precautions  against  cold.  He  will-  fear 
to  go  out  and  when  he  does  go  out,  he  will  put  on  chest  protectors,  scarfs, 
abdominal  binders,  etc.  Overheated  he  then  exposes  himself  to  cold 
more  readily  and  frequently  takes  cold.  All  these  disturbances  are 
usually  slight,  but  the  patient  will  always  exaggerate  them  to  an  unusu  al 
degree. 

A  very  frequent  form  of  hypochondria  is  the  one  pertaining  to  the  sex- 
ual function.  Impotence  is  a  common  complaint.  In  reality  there  is 
only  a  fear  and  anxiety  about  the  sexual  power.  In  the  majority  of  cases 
the  patients  are  capable  to  perform  the  act.  It  is  therefore  a  "psychic 
impotence."     Being  preoccupied  by  their  ideas,    they  look  for   causes 


460  HYPOCHONDRIA 

of  their  impotence,  and  should  masturbation  be  present  in  their  history, 
they  attribute  the  former  to  the  latter  and  believe  themselves  incurable. 
An  occasional  emission  will  reinforce  such  a  belief. 

Another  characteristic  feature  of  the  hypochondriacs  is  the  tendency 
to  speak  to  everybody  about  their  ills.  Not  infrequently  they  keep  accur- 
ate records  of  the  slightest  changes  in  their  condition.  One  of  my  patients 
used  to  send  me  every  fourth  day  a  most  detailed  account  of  his  hourly 
state  of  health.  Going  from  physician  to  physician,  from  hospital  to 
hospital,  they  carry  with  them  their  records  and  insist  upon  reading  them. 
During  the  examinations  they  watch  the  expression  of  the  physician's 
face  all  ready  to  interpret  in  their  own  way  the  least  change.  The  edu- 
cated hypochondriac  frequently  consults  medical  books,  looks  for  his 
symptoms  in  various  diseases  and  usually  in  the  most  incurable  affections. 
His  fear  and  anxiety  are  naturally  increased  and  the  more  he  examines 
himself  the  more  he  becomes  convinced  of  the  hopelessness  of  his  condition. 
Desperate  he  leaves  the  regular  physician,  procures  patent  medicines,  con- 
sults quacks,  takes  up  osteopathy,  faith  cure,  Christian  science,  etc.  Some- 
times he  keeps  on  taking  medicines  and  follows  at  the  same  time  some 
of  the  above  special  cures.  This  state  of  affairs  continues  months  and  even 
years. 

Diagnosis. — The  disease  is  frequently  confounded  with  neurasthenia. 
In  the  latter  there  is  the  typical  chronic  fatigue  manifested  upon  the  least 
mental  or  physical  exertion,  the  striking  irritability  upon  the  least  provoca- 
tion. In  hypochondria  these  symptoms  are  absent.  It  is  true  that  in 
some  cases  of  neurasthenia  there  may  be  present  some  hypochondriacal 
ideas.  In  the  majority  of  cases  each  of  these  affections  is  separately 
encountered  and  the  typical  pictures  characteristic  of  each  are  observed. 

A  hypochondriacal  condition  is  observed  as  an  early  stage  of  some 
forms  of  insanity.  The  qualitative  mental  changes  of  the  latter  are  not 
found  in  hypochondria. 

Etiology. — A  neuropathic  basis  is  commonly  observed.  Idleness, 
monotony,  absence  of  interest  in  any  special  pursuit  in  life,  sedentary 
occupations,  alcoholic  or  other  excesses,  lowering  of  the  general  nutrition, 
are  all  important  causes  of  hypochondria.  It  is  frequently  observed  in 
advanced  medical  students  and  young  practitioners.  It  is  also  observed 
in  those  individuals  who,  after  having  accumulated  large  means,  retire 
at  a  too  early  age  and  begin  to  lead  a  life  free  from  activity. 

Course.  Duration.  Prognosis  of  Psychoneuroses. — The  phenomena 
described  above  do  not  necessarily  accompany  the  psychoneurotic  indi- 
vidual through  his  entire  life.  They  are  but  episodic  manifestations. 
They  may  appear  at  any  time  upon  the  least  accidental  psychic  shock 


HYPOCHONDRIA  46 1 

or  after  some  intercurrent  disease.  They  disappear  after  an  appropriate 
treatment,  but  may  reappear  with  the  greatest  facility.  These  individuals 
possess  a  special  make-up  of  their  nervous  system,  whose  reaction  to 
external  and  internal  stimuli  is  different  from  that  of  normal  individuals 
and  demonstrates  an  extraordinary  emotionality,  impressionability,  ex- 
tremely prompt  response  to  the  slightest  stimulation.  Instability  of 
character,  of  disposition  and  of  mentality — are  the  chief  characteristics 
of  the  psychoneurotic  or  psychasthenic  individuals. 

Recovery  is  possible,  but  usually  the  condition  lasts  many  years. 
It  may  disappear  with  advanced  age. 

Treatment  of  Psychoneuroses.  Psychotherapy.  Psychoanalysis. — 
Before  psychotherapy  and  psychoanalysis  are  discussed,  a  few  words 
will  be  said  of  the  general  management  of  the  cases.  Psychoneurotic 
individuals  are  persons  with  a  specially  unstable  nervous  system.  Ap- 
propriate hygienic  measures  should  be  always  applied.  Careful  dis- 
crimination must  be  made  in  the  choice  of  occupation.  A  career  free  from 
great  mental  strain  and  from  multiple  obligations  should  be  chosen.  A 
simple  life,  regular  habits,  avoidance  of  alcohol  and  of  sexual  excesses, 
hydrotherapy,  outdoor  moderate  exercises,  are  essential. 

The  nature  of  the  manifestations  observed  in  psychoneurosis  is  a 
sufficient  indication  of  the  fact  that  mere  medications  or  physical  means 
will  fail  to  relieve  the  tormenting  thoughts  of  psychasthenic  individuals. 

The  chief  element  which  is  affected  is  the  psyche.  The  treatment  in 
such  cases  must  therefore  be  before  everything  else  psychic.  The  main 
object  consists  in  enabling  the  sufferer  to  overcome  the  distressing  thoughts 
by  means  of  mastering  his  will  power  and  thus  dislodge  the  obsessive 
thoughts  which  are  so  persistent  and  so  tenacious.  The  chief  aim  in 
psychotherapeutics  is  to  reach  the  point  where  the  patient  begins  to  gain 
conviction  of  the  curability  of  his  case.  As  soon  as  this  is  attained,  recov- 
ery follows  rapidly.  But  to  reach  that  conviction  requires  considerable 
effort  on  the  part  of  the  physician.  It  can  be  accomplished  by 
gradual  and  repeated  suggestive  influence.  Suggestion  can  be  exercised 
in  any  form  for  practical  purposes.  Thus  advantage  was  taken  of  this 
idea  by  various  sects  and  lay  persons.  The  history  of  Lourdes  in  France, 
of  Christian  Science,  of  hypnotism,  of  Emmanuel  movement,  as  well  as 
of  mesmerism  with  its  theory  of  magnetism — all  proves  that  suggestion 
has  given  even  with  the  unscientific  methods  some  good  results  in  some 
selected  cases.  The  scientific  conception  of  suggestive  methods  begins 
with  Bernheim  of  Nancy.  Suggestion  consists  of  forcing  into  the 
patient's  mind  the  idea  or  the  conviction  of  his  eventual  recovery.  This 
conviction  can  be  gained  in  two  ways:  either  blindly  or  by  the  way  of 


462  HYPOCHONDRIA 

reasoning.  In  the  first  case  the  patient  falls  entirely  under  the  healer's 
influence,  such  as  is  seen  in  hypnotism  or  in  the  methods  employed  by 
various  cults  or  religious  movements.  In  the  second  case,  viz.  with  the 
method  of  reasoning  the  conviction  is  gained  by  the  patient  after  one  or 
several  seances  of  logical  and  reasonable  demonstration  of  the  unfounded 
cause  of  anxiety  or  worry  over  ills  that  are  not  in  existence.  This  method 
is  persuasion.  It  is  rational  and  scientific.  We  have  here  an  exposi- 
tion of  facts  in  the  interpretation  of  which  the  patient  himself  partici- 
pates intelligently.  It  is  most  enduring  as  the  patient's  intelligence  is 
thereby  trained  in  the  method  of  observation.  A  hypochondriac,  for 
example,  complains  of  his  heart,  kidneys,  liver,  which  undergo  degen- 
eration in  his  judgment.  The  first  requisite  in  such  a  case  is  the 
necessity  of  the  patient's  confidence,  and  this  can  be  accomplished  by 
giving  the  patient  our  close  attention.  He  must  feel  that  we  are 
ready  and  anxious  to  hear  his  account  from  beginning  to  end,  that 
we,  as  a  friend,  are  interested  in  his  welfare.  We  must  exhibit  a 
certain  amount  of  sympathy.  Sincerity  and  actual  charitable  spirit 
must  be  present  in  the  physician.  The  next  step  is  to  teach  the  patient 
that  his  heart  is  not  diseased  by  teaching  him  to  count  his  own  pulse, 
by  pointing  out  the  absence  of  various  disorders  in  the  lungs,  kidneys. 
If  the  patient's  fixed  idea  has  a  relation  to  the  kidneys,  examine  his  urine 
in  his  presence,  demonstrate  the  absence  of  abnormal  elements  indicative 
of  a  renal  lesion.  Should  the  patient  actually  suffer  from  some  slight 
disturbances  in  the  domain  of  any  of  his  organs,  an  appropriate  treatment 
must  be  instituted.  Special  stress  must  be  laid  on  the  slightest  improve- 
ment, on  the  disappearance  of  symptoms,  so  that  the  patient  will  see  in  it 
each  time  a  new  reason  for  encouragement.  If  the  patient's  expression, 
tone  of  voice,  reveals  still  some  skepticism,  it  must  be  at  once  attacked  with 
gentle  vigor,  always  basing  oneself  on  facts  which  should  be  actually 
demonstrated  to  the  patient.  In  such  a  manner  the  patient  will  succumb 
to  evidences  brought  before  him  and  accept  our  statements  as  incon- 
trovertible. Considerable  tact  and  skill  must  be  displayed  by  the  phy- 
sician. By  tactful  and  encouraging  words  or  demeanor  he  will  be  able 
to  exercise  a  powerful  influence  in  his  attempts  to  dislodge  the  morbid 
fixed  ideas.  Psychotherapy  understood  in  its  proper  sense  is  useful  in 
management  of  all  ills,  but  finds  its  greater  field  of  usefulness  in  psycho- 
neuroses. 

Psychoanalysis. — In  spite  of  the  excellent  results  obtained  in  a  large 
number  of  cases  by  the  methods  of  persuasion  and  suggestion,  there  are 
nevertheless  instances  in  which  psychotherapeutics  are  insufficient  and 
sometimes  meet  with  total  failure.     A  new  psychological  method  has  been 


HYPOCHONDRIA  463 

developed  by  Breuer  and  especially  by  Freud,  which  enables  one  to  get 
a  deeper  insight  into  the  nature  and  origin  of  the  morbid  phenomena  of  the 
psychoneuroses.  It  is  based  on  the  principle  that  the  mental  processes  of 
an  individual  are  composed  of  two  separate  groups.  One  of  them  controls 
the  conscious  acts  of  daily  life.  The  other  group  constitutes  ideas  and 
wishes  which  occurred  to  us  some  time  ago,  but  which  the  conscious  ego 
endeavored  to  reject,  but  as  they  cannot  be  annihilated  totally,  they 
remain  in  a  latent  state  and  therefore  subconscious.  The  conscious 
personality  continuously  strives  to  absorb  the  latter  by  assimilation,  but 
when  it  is  incapable  to  do  so,  a  conflict  is  established  between  the  two 
groups  of  thoughts,  and  then  various  phenomena  characteristic  of  psy- 
choneuroses make  their  appearance.  Normally  the  conscious  ego  is 
victorious  in  the  conflict,  the  subconscious  ideas  are  assimilated  and 
destroyed,  but  if  the  latter  are  only  pushed  to  the  background,  they  will 
invariably  emerge  and  under  the  influence  of  any  exciting  cause  will 
manifest  themselves  in  some  morbid  act  or  morbid  tendency.  The 
latter  may  not  take  the  form  of  the  original  idea  or  wish,  but  some  other 
form  and  become  thus  merely  a  substitute. 

If  there  is  a  possibility  of  bringing  to  the  surface  the  subconscious 
mental  presentations  so  that  the  conscious  ego  will  thus  be  enabled  to  meet 
them,  get  full  control  over  them  and  assimilate  them,  the  morbid  mental 
phenomena  will  be  removed.  One  of  the  procedures  utilized  for  this  purpose 
is  the  free  association"  method.  The  patient  is  told  to  concentrate 
his  thoughts  on  his  special  psychoneurotic  disturbance  and  bring  to  his 
memory  all  possible  ideas  that  occur  to  him  at  that  time.  He  must  be 
reminded  not  to  select  only  those  ideas  that  in  his  opinion  are  proper  to 
relate  but  repeat  indiscriminately  every  insignificant  fact,  no  matter  how 
ridiculous  or  improper  it  may  appear.  In  the  mass  of  expressions  and 
sentences  uttered  by  the  patient  who  is  unburdening  himself  from  long 
forgotten  thoughts  the  so-called  pathogenetic  link  will  be  found.  It 
will  be  seen  that  the  patient's  suffering  is  but  the  outer  expression  of  an 
effect  produced  by  ideas  or  wishes  concealed  in  the  subconscious  world 
of  the  individual's  ego.  When  they  are  brought  to  light,  the  patient  sees 
them  one  by  one  passing  before  him,  he  gains  control  over  them  and  finally 
frees  his  personality  from  them. 

The  psychoanalysis  just  described  is  unquestionably  a  very  useful 
procedure.  Unfortunately  in  its  practical  applications  it  presents  some 
difficulties.  The  most  objectionable  among  them  is  that  it  consumes 
considerable  time  and  many  seances  with  the  patient  before  we  succeed 
in  having  him  unburden  himself  freely  from  all  his  thoughts,  especially 
those  of  a  disagreeable  nature  or  of  a  sexual  character.     Freud  claims  that 


464  HYSTERIA 

the  chief  causal  factor  in  all  psyche-neuroses  including  hysteria  is  always 
a  sexual  moment,  a  sexual  trauma  occurring  some  time  in  the  patient's 
life  even  in  childhood;  that  the  psychoneurotic  phenomena  are  the  result 
of  the  conflict  between  libido  and  sexual  repression  and  the  symptoms  are 
a  sort  of  as  compromise  between  these  two  psychic  streams. 

This  conception  of  a  sexual  factor  as  being  the  only  etiological  factor 
in  psychoneurosis  is  not  as  yet  accepted  by  all. 

F.  ANXIETY  NEUROSIS 

A  state  of  "anxiety"  is  found  in  every  form  of  psychoneurosis,  but 
there  the  anxiety  is  a  secondary  phenomenon  or  the  consequence  of  other 
disorders  of  the  psyche.  Freud  has  isolated  a  clinical  picture  in  which  all 
the  components  of  the  symptom-complex  are  grouped  around  the  main 
symptom  of  anxiety.  He  termed  it  "anxiety  neurosis."  He  places  it 
among  the  psychoneuroses.     The  symptoms  are  as  follows. 

(1)  General  Irritability. — Auditory  hyperesthesia  is  frequent.  The 
latter  causes  insomnia. 

(2)  Anxious  Expectation. — It  is  associated  with  a  pessimistic  view  of 
things.  The  patient  interprets  every  occurrence  as  an  unfavorable  omen 
to  himself.  This  is  the  most  important  symptom  of  the  neurosis.  The 
anxiety  may  develop  even  without  an  associated  idea,  such  as  fear  of  going 
insane  or  of  sudden  death,  or  else  it  may  be  connected  with  disturbances 
of  the  somatic  functions,  viz.  cardiac,  respiratory,  vasomotor,  gastro- 
intestinal, muscular,  vesical,  etc.  Freud  considers  this  syndrome  as  having 
nothing  to  do  with  neurasthenia,  contrary  to  the  custom  of  many  who 
consider  it  only  as  manifestations  of  neurasthenia.  There  can  be  no  doubt 
that  among  the  multiple  intermediate  and  unclassifiable  forms  of  psycho- 
neurosis and  nervousness  there  is  a  variety  in  which  the  chief  symptom 
is  "anxiety."  The  latter  predominates  in  the  clinical  picture  and  every 
other  symptom  is  subordinate  to  and  dependent  on  the  state  of  anxiety. 

Similarly  to  other  forms  of  psychoneurosis  Freud  considers  here 
sexual  influences  as  the  direct  etiological  factor.  The  treatment  there- 
fore should  be  psychoanalysis. 

HYSTERIA 

Hysteria  had  been  known  to  the  most  ancient  writers.  For  a  long 
time  it  was  considered  as  the  result  of  disturbed  function  of  the  genitalia 
and  especially  of  the  uterus.  Only  in  the  seventeenth  century  (Lepois 
and  Sydenham)  a  more  modern  view  was  proposed  and  the  cause  of 
hysteria  was  placed  in  the  brain.     With  the  advent  of  the  school  of 


HYSTERIA 


465 


Salpetriere  and  Charcot  at  its  head,  the  disease  was  given  the  first  proper 
interpretation  and  placed  upon  a  solid  scientific  basis.  Since  then  hysteria 
has  been  considered  as  a  well-defined  morbid  entity  which  presents  its 
special  physical  and  psychic  stigmata.  Charcot  said  that  it  was  essentially 
a  pyschic  malady  and  this  was  practically  accepted  by  all. 


Fig.  142  Fig.  143 

Figs.  142,  143. — Hysteric  Hemianesthesia.     (Original.) 
Anaesthetic  parts  are  shaded. 

Symptoms. — The   clinical   manifestations   of  hysteria  are:  Sensory, 
Motor,  Psychic  and  Visceral. 

I.  Sensory  Disturbances. — They  affect  the  general  sensations  as  well 
as  the  special  senses.  Anaesthesia  is  the  most  frequent  occurrence.  It 
is  characterized  by  its  mobility;  it  disappears  and  reappears;  it  changes 
in  its  intensity  under  the  influence  of  the  most  insignificant  cause.  The 
seat  of  hysterical  anaesthesia  presents  a  great  variability;  it  may  be  con- 
fined to  very  small  areas  on  the  limbs  or,  what  is  more  frequent,  to 
geometrical  segments  of  the  latter,  and  we  speak  then  of  glove-like  or 
stocking-like  anaesthesia.  Finally  an  entire  half  of  the  body  may  be 
affected  and  we  say  hemianaesthesia.  Among  other  regions  in  which  the 
loss  of  sensations  is  frequently  observed  is  the  pharynx.  The  anaesthesia 
may  not  be  absolute;  we  then  deal  with  a  diminished  sensibility  or  hypaes- 
thesia.  Loss  or  decreased  sensation  may  be  observed  in  regard  to  all 
forms  of  sensations — touch,  pain  and  temperature. 
3° 


466 


HYSTERIA 


Hysterical  anaesthesia,  generally  speaking,  does  not  inconvenience 
the  patient  and  has  to  be  looked  for.  Thus  the  patient  writes,  sews, 
takes  a  good  hold  of  fine  objects. 

The  anaesthesia  may  be  associated  with  or  substituted  by  hyperes- 
thesia (excessive  sensibility) .  The  latter  is  rarely  generalized;  it  is  usually 
confined  to  a  limb,  to  a  segment  of  a  limb,  to  a  very  small  area.  It  is  also 
characterized  by  its  mobility,  disappearance  and  reappearance.  There 
are  certain  zones  (hysterogenetic)  which  are  quite  constantly  found 
hypersesthetic,  viz.    the  spinal  column,  inframammary  regions,  sternum 


Fig.  144. — Hysteric  Anaesthesia. 
Ansesthetic  parts  are  shaded. 


Fig.  145. — Hysteric  Anaesthesia. 


and  the  groins.  Hysterical  headache  is  not  infrequent  and  may  assume 
the  form  of  migraine  or  syphilitic  headache  by  its  exacerbations  at  night. 
Very  frequently  the  pain  is  only  a  hyperesthesia  of  the  scalp.  Clavus 
is  a  characteristic  hysterical  pain;  it  is  confined  to  a  very  limited  area  of 
the  vertex  of  the  head.  Occipital,  nuchal,  intercostal,  coccygeal  pain 
may  occur  in  hysteria. 

Hysterical  pseudomeningitis  is  a  well-known  syndrome;  general 
malaise,  anorexia,  insomnia,  headache  and  delirium  are  present.  What 
will  differentiate  it  from  true  meningitis  is  the  persistent  absence  of  fever, 
also  the  pathological  state  of  the  cerebro-spinal  fluid  (see  the  latter) . 


HYSTERIA  467 

Paresthesias  in  the  form  of  coldness,  numbness,  burning,  etc.,  are 
quite  common  in  hysteria.  The  so-called  "globus  hystericus"  is  a  well- 
known  paresthetic  phenomenon.  It  consists  of  a  sensation  of  pressure 
in  the  throat  or  of  a  ball  passing  from  the  epigastrium  to  the  throat. 

The  special  senses  also  suffer  sometimes  in  hysteria.  Taste,  smell, 
audition  and  vision  are  not  infrequently  involved.  Perverted  hysterical 
taste  and  smell  are  well  known.  Hysterical  deafness  is  usually  unilateral. 
Special  mention  must  be  made  of  visual  disturbances. 

Total  amaurosis  is  rare,  but  contraction  of  the  visual  fields,  disturbed 
perception  of  color  and  disturbed  accom- 
modation are  quite  frequent.  As  to  the 
contracted  field,  it  is  concentric  in  the 
majority  of  cases;  it  is  more  often  bilateral 
than  unilateral.  The  latter  is  in  the  ma- 
jority of  cases  on  the  side  where  sen- 
sory losses  are  most  marked,  as  for  ex- 
ample in  case  of  hemiansesthesia.  Visual 
acuity  is  usually  intact.  To  this  symp-  Fig.  146.— Hysterical  Con- 
tom    is    sometimes    added  disturbance  of  ™A™™  °*  THE  Arm"    {GiUes  de 

la  1  oureue.) 

color    perception;    it    may  be  dyschroma- 

topsia  or  achromatopsia:  the  colors  are  either  confused  or  not   at  all 

recognized. 

II.  Motor  Symptoms. — Paralysis  and  contractures  are  very  frequent. 
Palsies  of  a  segment  of  a  limb,  of  an  entire  extremity  or  extremities — 
monoplegia,  hemiplegia  and  paraplegia — may  occur.  Hysterical  palsies 
and  contractures  vary  considerably  from  the  standpoint  of  their  onset, 
duration  and  mode  of  disappearance.  They  may  last  but  a  few  minutes 
or  persist  for  years.  Their  essential  feature  lies  in  complete  absence  of 
objective  signs  of  organic  nature.  In  hysterical  hemiplegia,  for  example, 
there  is  no  spasticity  of  the  muscles,  no  increased  or  abnormal  reflexes. 

•  As  to  contractures,  they  may  affect  only  the  fingers  or  the  toes,  a 
limb,  the  muscles  of  the  face  or  the  neck.  Blepharospasm  or  contracture 
of  the  orbicularis  palpebrarum,  facial  hemispasm  are  not  very  rare.  A 
spasm  of  the  eyelid  will  produce  a  ptosis  or  rather  a  pseudoptosis.  When 
the  head  is  thrown  back,  the  ptosis  disappears.  The  ocular  muscles  may 
similarly  be  affected.  An  insufficiency  of  the  internal  rectus  is  not 
infrequent.  Diplopia  is  sometimes  observed.  A.  Westphal  observed 
pupillary  rigidity  with  myosis  when  the  patient  concentrated  his  mind 
upon  it,  but  the  pupils  reacted  normally  when  his  mind  was  diverted. 
The  essential  feature  of  hysterical  facial  spasm  is  its  disappearance 
during  sleep.     Facial  palsy  is  sometimes  observed,  but  it  affectsjonly  the 


468  HYSTERIA 

lower  half  of  the  face.  It  is  recognized  by  this  sign  that  when  the  affected 
muscles  are  held  with  the  fingers  and  moved,  no  muscular  relaxation  is 
noticed,  but  on  the  contrary  a  certain  amount  of  resistance  is  felt.  Be- 
sides, the  palsy  may  become  ameliorated,  disappear  and  recur  almost 
every  day. 

Hysterical  torticollis  may  be  due  to  a  hysterical  palsy  or  contracture 
of  the  muscles  of  the  neck.     In  the  first  case  the  head  can  easily  be  placed  • 
in  a  straight  position,  but  it  returns  as  soon  as  it  is  abandoned.     The 
latter  form  may  disappear  suddenly  and  reappear. 

Contractures  may  also  affect  the  joints.  The  hip  and  the  knee 'are 
the  most  frequent  seats  of  this  condition.  Hysterical  coxalgia  can  be 
recognized  by  the  absence  of  swelling  and  of  increased  local  temperature, 
of  pain  when  the  great  trochanter  is  percussed  and  b)'  the  absence  of 
crepitation  and  subluxation.  Hysterical  individuals  may  be  affected 
with  choreiform  movements  or  present  special  tremors.  The  former  may 
simulate  Sydenham's  chorea  with  its  irregular  and  arhythmical  move- 
ments or  more  frequently  presents  rhythmical  movements  affecting  one 
limb,  the  face  or  the  neck  and  occurring  at  regular  intervals.  Sometimes 
the  movement  resembles  dancing  (saltatory  chorea),  or  swimming  (nata- 
tory chorea).  One  of  my  patients,  a  girl  of  twelve,  with  distinct  hysterical 
stigmata,  would  suddenly  jump  off  the  chair  and  reproduce  for  one  minute^ 
dancing  movements.  This  came  on  in  attacks,  five  or  six  a  day,  and  she 
suffered  from  it  eighteen  months. 

Hysterical  tremors  are  polymorphous  in  character.  They  may  fre 
slow,  rapid,  slight  or  pronounced.  The}'  may  be  oscillatory  or  of  a  large 
amplitude.  They  are  present  when  the  patient  is  at  rest  and  become 
increased  by  excitement. 

An  interesting  motor  phenomenon  occurring  in  hysteria  is  Astasia- 
abasia.  It  consists  of  a  functional  impotence  in  gait  and  station;  the 
patient  cannot  stand  and  walk,  but  when  seated  or  bed-ridden  he  is  able 
to  perform  all  movements  with  his  limbs.  Various  degrees  of  this  motor 
disturbance  may  be  present,  from  absolute  inability  to  preservation  of 
some  movements. 

III.  Psychic  Symptoms,  (a)  Suggestibility. — The  most  essential  and 
characteristic  feature  of  hysterical  individuals  is  suggestibility.  They' 
are  easily  influenced  to  change  their  thoughts,  to  do  certain  acts,  to  acquire 
certain  sensations  in  the  general  sensorium  or  in  the  sphere  of  the  special 
senses,  to  execute  or  to  adopt  certain  motor  phenomena.  Experimental 
palsies,  anaesthesias,  contractions,  modifications  of  the  personality  are 
the  best  illustration.  The  hysterical  persons  are  not  conscious  of  the 
suggested  act,  they  do  not  understand  it,  they  do  not  connect  it  with 


HYSTERIA  469 

their  own  personality;  the  suggested  ideas  develop  in  them  automatically 
without  the  controlling  power  of  the  will.  For  the  same  reason  self- 
suggestion  is  also  observed ;  they  have  a  remarkable  tendency  to  reproduce 
what  they  have  once  seen  or  heard  because  of  extreme  sensibility  and 
impressionableness  of  their  psychic  centers,  so  that  real  hallucinations  are 
formed.  In  a  case  reported  by  me  in  the  American  Journal  of  the  Medical 
Sciences,  April,  1906,  numerous  psychic  phenomena  of  autosuggestion 
developed  under  the  influence  of  reading  or  seeing. 

(b)  Amnesia. — Disturbance  of  memory  is  a  frequent  occurrence  in 
hystericals.  Variability  of  answers,  untruthfulness,  contradiction, 
capriciousness,  inconsistencies  in  their  mode  of  living  and  conduct  are 
the  result  of  temporary  amnesia.  The  loss  of  memory  may  be:  localized 
when  events  of  a  certain  period  are  forgotten;  systematized,  when  several 
events  concerning  several  persons  or  several  periods  are  forgotten;  general, 
when  the  hysterical  forgets  his  entire  past  life  (see  Sidis  and  Goodhart 
on  Multiple  Personality).  A  person  thus  affected  may  lose  all 
remembrance  of  his  previous  life;  he  possesses  a  new  personality.  In 
some  cases  the  patient  passes  through  two  or  more  different  short  periods 
of  life  as  two  or  more  personalities  (double  consciousness)  and  one  is  not 
aware  of  the  other,  so  that  the  lives  the  two  personalities  lead  may  be 
diametrically  opposite  to  each  other.  (See  case  of  Mary  Reynolds — 
double  consciousness  by  Weir-Mitchell  and  my  case  reported  in  the 
American  Journal  of  Medical  Sciences,  1906.) 

(c)  Disturbance  of  Speech. — Mutism  occurs  in  hysteria.  The  patient 
is  not  only  unable  to  utter  words  in  a  loud  or  low  voice,  but  is  also  unable 
to  emit  a  sound,  although  the  movements  of  the  lips  and  tongue  are  well 
preserved.  Stuttering  may  also  occur  in  hysteria,  but  it  is  different  from 
the  usual  ^stuttering  acquired  in  childhood. 

In  the  latter  case  the  patient  is  able  to  make  himself  understood.  He 
pronounces  whole  sentences,  but  stutters  especially  in  pronouncing 
t certain  letters,  such  as  m,  t,  b.  After  the  stoppage  is  overcome,  he  goes 
on  speaking  clearly  until  again  the  special  letters  interrupt  him.  More- 
over when  the  patient  speaks  very  slowly  and  deliberately,  the  stuttering 
may  disappear.     The  stuttering  is  particularle  noticeably  in  rapid  speech. 

Hysterical  stuttering  is  present  not  only  for  special  letters,  but  it  is  con- 
tinuous. The  patient  stutters  at  every  word  or  syllable  and  during  his 
entire  conversation.  The  words  are  pronounced  by  him  slowly  as  he 
has  difficulty  in  emitting  all  verbal  sounds.  His  entire  speech  is  slow  and 
the  syllables  are  pronounced  with  prolonged  effort.  Finally  hysterical 
stuttering  occurs  after  a  violent  emotion,  such  as  railway  accident,  etc. 

Aphasia  is  not  frequent  in  hysteria.     Motor  and  sensory  aphasia 


470  HYSTERIA 

have  been  observed.  They  presented  all  the  typical  features  of  genuine 
aphasia,  except  the  onset,  course  and  termination.  Hysterical  aphasia  may 
occur  after  an  emotion,  imitation  or  suggestion.  It  is  variable  in  its 
course,  disappears  and  reappears  and  always  ends  in  recovery.  Agraphia 
in  association  with  aphasia  has  also  been  recorded.  It  has  the  same 
characteristics  as  aphasia. 

(d)  Somnambulism.  Catalepsy.  Lethargy. — The  first  consists  of  a 
sleeping  state,  during  which  the  patient  leaves  the  bed  and  walks  around. 
In  such  a  manner  he  is  likely  to  walk  through  the  window  and  fall  down 
on  the  roof  or  on  the  street.  Catalepsy  is  characterized  by  a  rigidity  of 
one  or  several  extremities  which,  placed  in  a  certain  position,  may  remain 
so  for  hours.  The  patient  hears  what  is  going  on  around  him,  but  unable 
to  control  himself;  he  is  probably  under  the  influence  of  a  hallucination. 
External  stimulations  (cold  water,  electricity)  frequently  arouse  the 
patient.  Lethargy  is  characterized  by  a  sleep  which  comes  on  suddenly 
and  during  which  the  patient  reacts  to  external  stimulation;  there  is  also 
no  relaxation  of  the  muscles,  but  some  rigidity;  the  masticator  muscles 
are  contracted,  the  eyelids  are  animated  with  a  tremor.  When  the 
patient  awakes,  he  has  no  recollection  of  the  attack  of  sleep. 

(e)  Hysterical  Fainting. — With  or  without  premonitory  symptoms 
the  patient  suddenly  feels  fainting  and  falls.  The  eyes  are  half-closed. 
The  hands  are  animated  with  slight  twitchings.  The  face  is  pale,  but 
the  heart  beats  are  normal. 

(/)  Hysterical  Paroxysms  (Major  Hysteria  of  Charcot). — They 
remind  of  epileptiform  seizures,  but  they  differ  essentially  from  the 
latter.  An  attack  is  usually  preceded  by  prodromal  symptoms  which 
are  of  a  psychic  order.  The  patient  becomes  sad,  depressed  or  else  very 
excitable;  sometimes  he  has  visual  or  auditory  hallucinations.  An  aura 
is  present, but  it  is  slow  in  coming  on;  it  is  usually  the  well  known  "globus 
hystericus"  (see  above).  Then  follows  the  attack.  The  patient  becomes 
unconscious  or  rather  semiconscious,  as  in  falling  he  rarely  injures 
himself,  and  in  some  cases  he  places  himself  comfortably  before  he  is 
seized  with  the  convulsions.  The  latter  are  at  first  tonic.  The  entire 
body  is  tetanized:  the  respiration  stops,  the  arms  and  legs  are  stretched 
out,  the  face  is  cyanotic.  Rapidly  the  clonic  contractions  appear.  Unlike 
those  of  epilepsy  they  are  of  wide  range  and  last  longer.  The  body 
assumes  all  sorts  of  attitudes.  Contortions  characterize  this  phase  of  the 
attack.  Flexion  or  extension  of  the  trunk  and  of  the  limbs,  throwing  the 
legs  in  all  directions,  rolling  the  body  from  one  side  to  another,  position 
of  opisthotonos,  grimaces  of  the  face — these  are  the  various  positions 
rapidly  succeeding  each  other.     At  the  same  time  the  patient  screams, 


HYSTERIA 


471 


cries  or  else  laughs,  tears  his  clothing,  etc.  Under  the  influence  of  hallu- 
cinations accompanying  this  period  the  patient  soon  develops  passionate 
attitudes.  They  are  expressed  in  the  gestures  and  the  display  of  the 
muscles  of  the  face.  The  images  he  sees  in  his  dream  express  alternately 
sadness,  anger,  terror,  joy  or  ecstasy.  In  my  case  referred  to  above  the 
patient  stood  in  the  middle  of  the  room  in  the  attitude  of  an  orator, 
reciting  a  pathetic  story  which  she  read,  ges- 
ticulating, raising  and  lowering  her  voice, 
crying  and  laughing. 

Gradually  the  violent  movements  cease, 
relaxation  takes  place  and  the  patient  enters 
into  the  last  period  of  the  paroxysm  which 


Fig.  147. — Hysterica!   Paroxysm. 
{Charcot.) 


Opisthotonos.  Fig.    148. — Passionate  Atti- 

tude in   a   Hysterical  Attack. 
(Gilles  dc  la  Tourette.) 


is  characterized  by  a  state  of  delirium.  He  talks  in  a  low  tone  of  voice, 
speaks  of  a  certain  recent  event,  which  has  made  a  special  impression  on 
him.  In  some  cases  instead  of  a  delirium  the  attack  may  terminate  by 
an  out-burst  of  laughing,  singing  or  crying. 

Little  by  little  and  sometimes  suddenly  the  patient  becomes  silent, 
awakens  and  the  attack  is  over.  The  patient  may  feel  somewhat  fatigued, 
but  he  does  not  present  the  profound  exhaustion  following  an  epileptic 
seizure.  An  attack  usually  lasts  from  ten  to  fifteen  minutes,  but  the 
periods  of  grand  movements  and  of  delirium  may  last  hours. 

The  paroxysms  do  not  always  present  the  typical  picture  just  described. 
They  may  vary  considerably.  In  some  cases  the  convulsive  movements 
begin  suddenly  without  an  aura.  In  others  the  delirium — as  a  final  phase 
— is  absent.  In  still  others  there  are  only  tonic  movements.  The  move- 
ments themselves  may  be  prolonged  or  unusually  brief.  They  may  be 
limited  to  a  certain  limb  or  portion  of  the  body.     In  one  of  my  recent 


472 


HYSTERIA 


Fig.  149. — Hysterical  Paroxysm.    Are  de 
Cercle.     (Gilles  de  la  Tourelte.) 


cases  the  convulsions  simulated  Jacksonian  epilepsy,  as  they  were  confined 
to  the  right  arm  and  leg. 

The  hysterical  paroxysms  are  rarely  isolated.  They  repeat  them- 
selves sometimes  at  regular  intervals^upon  the  least  emotion.  Sometimes 
one  attack  follows  another  and  this  condition  may  last  hours  and  days. 
The  patient  is  then  in  status  hystericus.  Hysterical  paroxysms  may  be 
associated  with  epileptic  seizures.     Such  a  combination  is  called  hystero- 

epilepsy.  The  latter  should,  how- 
ever, not  be  considered  as  a 
special  entity,  as  it  is  only  an 
association  of  two  independent 
neuroses,  each  with  its  character- 
istic symptoms. 

The  So-called  Hysterical  In- 
sanities.— The  last  two  stages  of 
a  typical  hysterical  paroxysm 
can  be  placed  under  this  heading. 
The  delirium  and  the  hallucina- 
tions under  which  the  patient 
is  laboring  at  that  time  render 
him  totally  irresponsible.  Hysteria  may  simulate  various  mental  affec- 
tions. Attacks  of  depression  or  else  of  excitement,  persecutory  or 
other  delusions  associated  with  hallucinations  may  give  the  impression 
of  classical  psychoses.  Hysterical  melancholia,  hysterical  mania,  hys- 
terical paranoia  have  been  described,  but  they  are  not  genuine  forms 
of  mental  alienation.  A  careful  analysis  of  the  symptoms  will  show  that 
attacks  of  delirium,  of  depression,  of  excitement  and  even  periods  of  appa- 
rent mental  derangement  are  only  episodes  in  the  life  of  hysterical  in- 
dividuals; they  are  equivalents  of  ordinary  hysterical  paroxysms,  just  as 
psychic  phenomena  take  sometimes  the  place  of  ordinary  epileptic 
seizures.  The  course,  character,  duration  and  the  consequences  of  the 
psychic  manifestations  are  not  at  all  those  of  the  typical  psychoses;  they 
are  all  a  sort  of  transformation  of  ordinary  hysterical  paroxysms  and  they 
all  may  be  the  result  of  autosuggestion.  The  conception  of  hysterical 
insanities  as  morbid  entities  is  erroneous. 

IV.  Visceral  Disturbances. — The  larynx  may  be  the  seat  of  various 
symptoms.  Aphonia  is  not  infrequent,  it  comes  on  suddenly;  patient  is 
unable  to  raise  his  voice;  he  only  whispers.  The  laryngoscope  reveals 
total  absence  of  palsy  of  laryngeal  muscles.  Various  laryngeal  noises 
are  also  observed.  Hiccough  is  a  familiar  phenomenon.  It  develops 
suddenly  and  may  disappear  suddenly.     It  occurs  in  attacks  lasting  a 


HYSTERIA 


473 


certain  number  of  days  or  weeks.  It  is  usually  very  loud  and  may  resemble 
the  barking  of  a  dog.  Hysterical  cough  is  paroxysmal.  It  usually  appears 
upon  emotion  and  disappears  when  the  patient's  mind  is  diverted  toward 
a  special  subject.  It  disappears  when  the  patient  is  asleep.  Hysterical 
dysphagia  consists  of  an  intermittent  spasm  of  the  pharynx  and  oesophagus. 
If  it  is  continued,  it  will  lead  to  inanition.  The  same  can  be  said  of 
hysterical  anorexia,  which  may  become  associated  with  attacks  of  vomit- 
ing. These  gastric  disturbances  may  be  only  temporary  or  persistent. 
In  the  latter  case  they  are  quite  serious. 

Tympanitis  is  sometimes  observed;  it  may  be  partial  or  generalized. 
It  may  simulate  pregnancy.     Hysterical  pseudo-peritonitis  may  occur. 


Fig.  150. — Hysteria.     Symmetrical  Erythematous  Patches  on  the  Chest  which 
Developed  Rapidly  after  a  Violent  Emotion. 


It  may  be  accompanied  by  hyperesthesia  of  the  abdomen,  vomiting, 
constipation,  but  fever  is  absent.  It  may  also  simulate  appendicitis. 
In  one  of  my  cases  the  patient  was  operated  without  my  knowledge  and 
the  appendix  was  found  absolutely  normal.  Shortly  after  the  operation 
the  hyperassthesia  of  the  same  region  returned. 

Hysterical  anuria  and  dysuria,  also  retention  of  urine,  are  occasionally 
observed.  One  of  my  female  patients,  after  a  series  of  shocks — death 
and  diseases  in  the  family — developed  anuria  which  alternated  with  re- 
tention of  urine.  Polyuria  is  not  an  infrequent  phenomenon.  Sexual 
function  may  be  altered.  Either  there  is  an  increase  or  diminution  of 
sexual  appetite;  sometimes  there  are  perversions  of  sexual  desire. 

Vasomotor  disturbances  are  seen  as  sudden  flushing  or  pallor  of  the 
skin,   dermography.     The  latter   consists    of    persistency  of  a  red  line 


474  HYSTERIA 

on  the  skin  when  the  latter  is  slightly  irritated.  Erythematous  patches 
following  a  violent  emotion  may  occur.  Various  spontaneous  hemorrhages 
(independently  of  cases  of  malingering  to  which  hysterical  patients  are 
subject)  may  be  observed:  ecchymosis,  epistaxis,  hematemesis,  hematuria, 
hemoptysis  (Fig.  150).  They  are,  however,  rare.  The  menstruation  may 
be  disturbed.  Hysterical  fever  cannot  be  denied.  It  develops  sponta- 
neously and  comes  down  without  any  treatment.  The  oscillation  is  of  very 
wide  range.  It  may  be  present  without  the  usual  accompanying  symp- 
toms, viz.  headache,  rapid  pulse,  thirst  or  burning  of  the  skin.  Hysterical 
fever  is  rare.     Malingering  should  always  be  excluded. 

Trophic  disturbances  occasionally  occurring  in  hysteria  are :  oedema, 
ulcerations,  sudden  falling  out  or  graying  of  the  hair.  I  have  ob- 
served a  remarkable  case  of  gangrenous  patches  on  the  chest  and  arms 
which  leave  no  doubt  as  to  their  hysterical  nature.  They  developed 
rapidly  after  a  fright,  began  with  a  simple  erythema,  persisted  for  a  long 
time  without  spreading,  but  commenced  to  improve  and  finally  recovered 
when  electricity  was  applied  a  few  times.  Another  shock  occurred, 
again  a  few  patches  appeared  and  again  they  disappeared  when  electricity 
was  resorted  to. 

Course,  Duration,  Prognosis. — The  above  described  sensory,  motor 
psychic  and  visceral  symptoms  are  not  always  present  in  every  case. 
There  are  mild  and  severe  cases.  The  disease  usually  runs  a  chronic 
course.  The  symptoms  develop  gradually  under  ordinary  circumstances. 
They  may,  however,  develop  rapidly  when  a  shock,  an  emotion,  are  the 
etiological  factors.  The  essential  characteristic  feature  of  hysteria  is  the 
great  variation  in  the  manifestations.  Some  symptoms  disappear,  but 
may  reappear  upon  the  least  emotion;  others  persist.  The  prognosis  as 
to  life  is  good,  except  in  cases  of  marked  visceral  disturbances,  as  spasms 
of  the  larynx  or  dysphagia  or  persistent  anorexia. 

Hysteria  as  a  rule  is  a  curable  affection,  but  in  some  cases  it  may  last 
indefinitely.  The  duration  and  the  prospects  of  recovery  depend  upon 
the  circumstances,  surroundings,  mode  of  living  and  the  treatment. 

The  hysterical  paroxysms  are  difficult  to  combat,  they  are  exceedingly 
stubborn.  The  contractures  are  also  difficult  to  cure.  In  children  the 
disease  is  less  persistent  than  in  adults  and  the  usual  stigmata  are 
frequently  absent.  The  symptoms  are  rather  single;  either  mutism,  or  a 
paralysis,  or  a  contracture,  etc. 

Diagnosis.- — In  making  a  diagnosis  of  hysteria,  first  of  all  organic 
diseases  should  be  eliminated.  Hysterical  palsies,  for  example,  may 
simulate  hemiplegia  or  monoplegia.  The  following  are  the  differential 
signs  of  the  two  forms  of  paralysis. 


HYSTERIA 


475 


Organic  Hemiplegia 
i.  Spasticity  of  the  affected  limbs. 


2.  Tendon    reflexes    abolished   im 
mediately    after    the    onset,    but 
later  exaggerated. 

3.  Babinski  sign  present. 

4.  Oppenheim's     and    paradoxical 

reflexes  present. 

5.  Ankle-clonus  present. 

6.  Voluntary    movements    on    the 

paralyzed  side  are  abolished. 

7.  Abdominal  and   cremasteric  re- 

flexes are  usually,  esepcially 
at  the  beginning,  diminished 
or  abolished. 

8.  The    course    is     regular:     spas- 

ticity follows  flaccidity;  im- 
provement is  gradual. 


Hoover's  "opposition  move- 
ment" phenomenon  is  present. 
(When  the  patient  makes  an 
effort  to  raise  the  paralyzed 
leg,  there  is  a  very  energetic 
"opposition  movement"  in 
the  other  leg;  a  hand  placed 
between  the  heel  of  the  sound 
foot  and  the  bed  will  appre- 
ciate the  degree  of  force  dis- 
played during  the  opposition 
movement). 


Hysterical  Hemiplegia 

1.  Flaccidity  of  the  affected  limbs. 
If  some  rigidity  is  present,  a  volun- 
tary resistance  during  passive 
movements  is  felt  by  the  hand  of 
the  examiner,  showing  therefore 
the  patient's  ability  to  oppose  the 
movements.  In  organic  hemi- 
plegia the  patient  is  unable  to  resist. 

2.  Tendon  reflexes  intact. 


3.  Babinski  sign  absent. 

4.  Oppenheim's     and     paradoxical 

reflexes  absent. 

5.  Ankle-clonus  absent. 

6.  Voluntary    movements    on    the 

paralyzed  side  are  not  totally 
abolished. 

7.  Abdominal  and  cremasteric  re- 

flexes are  normal. 


The  course  is  irregular:  flac- 
cidity or  some  rigidity  remains; 
the  disturbances  may  change 
from  time  to  time,  become 
aggravated  or  ameliorated  or 
else  last  a  very  short  time. 

Hoover's  "opposition  move- 
ment" phenomenon  absent. 
A  hand  placed  between  the 
heel  of  the  sound  foot  and 
bed  will  fail  to  find  the  counter- 
pressure  of  the  latter. 


476  HYSTERIA 

Sicard  has  recently  called  attention  to  the  following  phenomenon. 
If  an  elastic  bandage  is  applied  tightly  to  the  paralyzed  and  non-paralyzed 
symmetrical  limb  at  equal  distances  from  the  periphery  (usually  in  the 
middle  of  the  limb) ,  it  is  observed  that  in  functional  cases  the  vaso-motor 
disturbance  will  be  equal  on  both  sides,  but  unequal  in  organic  cases. 

For  other  additional  reflexes  observed  in  organic  and  absent  in  hyster- 
ical hemiplegia  see  pages  92,  93,  94. 

Hysterical  paraplegia  will  be  recognized  by  the  integrity  of  the  reflexes, 
absence  of  Babinski's,  Oppenheim's  and  paradoxical  signs  or  ankle-clonus. 

Hysterical  contractures  are  usually  sudden  in  onset,  disappear  under 
suggestion  and  under  general  anaesthesia. 

Hysterical  paroxysms  (see  above)  are  differentiated  from  epileptic 
seizures  by  the  following  signs. 

Epilepsy  Hysteria 

1.  Onset   abrupt.     Patient   drops     1.  Onset  after  an  emotion.     Patient 

unconscious.  feels  the  oncoming  attack  and 

takes    measures    not    to    fall. 
Semi-consciousness. 

2.  Patient  bites  his  tongue.  2.  Patient  does  not  bite  the  tongue. 

3.  Involuntary  micturition  or  de-      3.  Xo    involuntary    micturition    or 

fecation.  defecation. 

4.  Convulsive       movements       of     4.  Convulsive   movements    of   wide 

small  range.  range. 

5.  Pallor  of  the  face  after  a  seizure.      5.  No  pallor  of  the  face. 

6.  Exhaustion  and  profound  sleep     6.  Either  immediate  return  to  usual 

after    a    seizure;    also    some  occupation  or  a  superficial  sleep 

mental   hebetude   for    hours  after  an  attack.     Mentality 

following.  clear. 

Sensory  disturbances  are  observed  in  organic  diseases  of  the  nervous 
system  as  well  as  in  hysteria.  The  characteristic  features  of  hysterical 
anaesthesia  was  sufficiently  described  in  the  symptomatology  (anaesthesia  of 
segmentary  or  hemiplegic  form).  Hysterical  neuralgia  will  be  recognized 
by  the  absence  of  tenderness  of  the  nerve-trunks  characteristic  of  true 
neuralgia.  Hysterical  headache  may  sometimes  simulate  headache  caused 
by  a  cerebral  tumor,  but  the  mental  condition  accompanying  the  latter 
dullness,  apathy,  etc.)  in  addition  to  other  objective  symptoms  will  reveal 
the  true  nature  of  the  headache.  Besides,  the  failure  of  pain  to  affect 
the  general  condition  of  the  patient  is  strongly  in  favor  of  hysteria.  Pain 
caused  by  an  organic  disease,  such  as  deep-seated  tumors  and  other  affec- 
tions, produces  usually  a  reaction  on  the  general  health. 


HYSTERIA  477 

Etiology. — The  disease  is  very  frequent.  It  occurs  not  only  in  adults, 
but  also  in  children  and  aged  individuals.     Both  sexes  are  subject  to  it. 

The  causes  are  predisposing  and  exciting. 

Predisposing. — Heredity  plays  an  important  role.  Functional  nervous 
diseases,  insanities,  alcoholism,  tuberculosis,  syphilis  in  the  parents,  are 
predisposing  factors  in  the  offspring.  Excesses  of  all  sorts,  unhygienic 
mode  of  living,  sedentary  life,  certain  occupations  accompanied  by  a  great 
mental  strain  and  anxiety  are  all  predisposing  causes  for  hysteria. 

Among  the  exciting  causes  the  most  common  is  a  shock,  an  emotion. 
It  frequently  follows  accidents.  Traumatic  hysteria  is  a  very  well-known 
condition.  In  such  a  case  hysteria  is  due  exclusively  to  the  fright  and  to 
the  psychic  shock.  In  the  chapter  on  traumatic  neuroses  the  subject 
will  be  considered  in  detail.  In  the  chapter  on  "Psychic  Symptoms"  it 
was  mentioned  that  the  chief  characteristic  of  hysteria  is  "suggestibility." 
Suggestion  therefore  is  also  one  of  the  causes  of  hysteria.  It  may  be  in- 
duced sometimes  by  the  examining  physician,  if  he  is  not  careful  in  his 
procedures.  In  examining,  for  example,  for  sensory  disturbances,  care 
must  be  taken  not  to  speak  to  the  patient  of  the  desired  element  of  investi- 
gation. An  excellent  precaution  is  to  have  the  patient  blindfolded  during 
the  examination  and  not  to  ask  any  questions  of  her  while  you  test  with 
a  pin  various  areas  of  the  skin.  There  is  no  doubt  that  she  will  make  some 
defense  movement  if  the  prick  causes  pain.  Or  else  blindfolded  she  may 
be  asked  to  say  ''yes"  the  moment  she  feels  pain  or  the  touch  of  an  object. 
Moreover  she  should  never  be  spoken  to  of  possibilities  of  loss  of  power 
in  one  or  more  limbs  or  segment  of  a  limb,  or  loss  of  memory,  of  a  grave 
outlook,  etc.,  etc.  The  patient  may  be  suggested  various  disturbances 
of  function  in  the  motor,  sensory,  vaso-motor,  vegetative  and  psychic 
spheres  with  great  facility. 

Protracted  diseases  of  any  nature  may  be  a  cause  of  hysteria.  Intoxi- 
cations (alcohol,  lead,  mercury,  morphin,  C02)  are  also  among  the  exciting 
causes  of  hysteria.  Hypnotism,  especially  when  repeated,  develops 
hysteria.  The  provocative  factors  of  hysteria  are  therefore  multiple. 
However  it  should  be  borne  in  mind  that  this  malady  can  develop  only 
in  individuals  who  are  predisposed  to  it.  A  special  morbid  basis  is  essen- 
tial :  a  physical  or  mental  shock  will  have  no  special  effect  on  an  individual 
free  from  hereditary  taint,  but  will  disturb  considerably  the  nervous  sys- 
tem of  a  person  whose  make-up  is  morbid  through  heredity  or  otherwise. 

Nature  of  Hysteria. — At  the  present  time  two  views  are  held  concern- 
ing the  nature  of  hysterical  phenomena,  viz.  the  so-called  psychological 
and  physiological. 

According  to  the  first,  hysteria  is  a  psychic  disease.     Charcot,  Moebius, 


47§  HYSTERIA 

Strumpell,  Bernheim  and  Janet  see  in  hysterical  manifestations  either 
disturbance  of  the  faculty  of  mental  representation  or  autosuggestion 
(see  above),  or  else  double  or  multiple  personality;  finally  a  narrowing 
of  the  field  of  conscience.  Anaesthesia,  the  most  important  according  to 
Charcot  of  all  stigmata,  may  be  explained  by  Janet's  "restrictions  of  the 
field  of  consciousness."  The  hysterical  patient  perceives  only  a  small 
number  of  sensations  and  gradually  they  may  entirely  disappear  and  he 
thus  becomes  anaesthetic.  For  the  same  reason  memories  of  movements 
may  be  lost,  hence  hysterical  paralysis.  In  astasia-abasia,  for  example, 
the  field  of  consciousness  is  so  limited  that  the  patient  loses  the  association 
of  mental  operation  only  for  standing  or  walking,  but  for  no  other  act. 
Hysterical  paralysis  is  sometimes  due  to  a  suggestion.  The  patient 
adopts,  for  example,  the  idea  of  not  being  able  to  walk.  This  idea  may  re- 
main in  the  subconscious  state  without  the  patient's  knowing  it.  Fixed 
idea  is  a  very  important  element  in  hysteria.  It  plays  a  predominant  role 
in  the  fife  of  the  hysterical  individual :  other  ideas  and  experiences  have  no 
restraining  influence  on  the  fixed  idea.  Consequently  suggestion  is  a 
very  potent  factor  when  ideas  are  forced  on  a  hysterical  person  by  another 
person.  Such  is  the  case  in  hypnotism.  There  is  introduced  into  the 
patient's  mind  the  idea  of  the  hypnotizer's  personality,  which  finally 
becomes  predominating.  It  is  therefore  evident  that  what  character- 
izes especially  the  hysterical  individual  is  the  want  of  mental  synthesis, 
the  inability  of  grouping  individual  ideas.  He  will  attach  himself  to 
one  fixed  idea  and  the  others  will  remain  in  a  subconscious  state.  Hyster- 
ical paroxysms  cannot  be  totally  explained  by  the  conception  of  "restric- 
tion of  the  field  of  consciousness."  It  is  true  that  a  paroxysm  is  the  result 
of  the  workings  of  some  predominating  idea  which  was  brought  on  by 
other  secondary  ideas  of  past  experiences.  Nevertheless  during  a  hyster- 
ical attack  consciousness  has  disappeared  and  consequently  restriction 
of  the  field  of  consciousness  is  not  altogether  applicable  here. 

According  to  the  physiological  theory  advanced  by  Sollier,  the  hyster- 
ical stigmata  or  paroxysms  are  of  purely  physical  nature.  Here  the 
primary  disorder  is  a  functional  disturbance  of  the  entire  brain  or  only  of 
some  of  its  centers.  The  sensory,  motor,  psychic,  trophic,  visceral  and 
circulatory  phenomena  will  depend  upon  the  centers  involved.  An  in- 
hibition of  the  latter  produces  the  corresponding  symptoms. 

In  the  chapter  on  Psychoanalysis  (page  462)  the  ideas  of  Breuer  and 
Freud  on  the  origin  of  Psychoneuroses  were  discussed  in  detail.  These 
two  authors  consider  also  hysteria  as  a  form  of  psychoneurosis.  They 
argue  that  the  various  physical  manifestations  of  hysteria  are  only 
substitutions  for  the  mental  disturbances,  stress  and  unpleasantness  (to  all 


HYSTERIA  479 

of  which  they  give  the  name  "affect")  created  by  past  experiences.  The 
latter  are  never  annulled  but  remain  in  the  subconscious  mind.  The 
distress  produced  by  these  latent  thoughts  which  are  continuously  at 
work,  must  be  relieved  and  this  is  frequently  done  in  hysteria  through 
some  physical  occurrence  as  a  substitute.  Breuer  and  Freud  state  that 
this  power  of  "conversion"  of  mental  disturbances  into  physical  manifes- 
tations is  characteristic  of  hysteria.  When  the  conversion  does  not  take 
place,  the  result  will  be  evidenced  in  various  phenomena  characteristic 
of  psychoneuroses  such  as  obsessions,  phobias,  etc.  (see  Psychasthenia.) 
By  reason  of  the  efforts  of  the  patient  to  relegate  the  painful  ideas  into 
subconsciousness,  Breuer  and  Freud  call  all  psychoneuroses  as  "defense- 
neurosis."  In  all  psychoneuroses  they  find  that  the  repressed  disagree- 
able thoughts  are  always  of  sexual  nature.  At  some  time  during  the 
patient's  life  even  in  infancy  a  sexual  shock  of  some  kind  occurred.  The 
contention  as  to  hysterical  phenomena  being  the  result  of  sexual  ex- 
periences, hidden  in  the  subconscious  world  is  not  accepted  by  all.  The 
psycho-analytic  method  of  treatment  of  hysteria  is  based  on  bringing  to 
light  all  concealed  and  repressed  ideas  and  thus  have  them  asimilated  by 
the  conscious  ego  (see  chapter  on  Psychoanalysis). 

Recently  Babinski  {Semaine  Medicale,  1909)  proposed  a  new  revision 
of  Hysteria,  from  the  symptoms  of  which  he  removed  some  manifestations 
which  had  been  and  are  still  considered  by  many  as  belonging  to  the  great 
neurosis.  Dismembered  in  this  manner  Hysteria  is  called  by  Babinski 
"pithiatisme."  In  the  first  place  he  believes  that  in  a  great  many  cases 
the  phenomena  so-called  hysterical  are  the  result  of  malingering.  In  the 
next  place  a  number  of  symptoms  which  are  not  brought  on  by  suggestion 
are  fictitious,  for  example :  fever  and  anuria.  In  the  third  place  symptoms 
such  as  erythema,  ecchymosis,  ulcerations,  gangrene,  oedema — are  in- 
tentionally brought  on;  they  are  fraudulent,  they  are  the  result  of  local 
applications  used  by  the  patient  for  the  purpose  of  arising  sympathy  or 
otherwise. 

Babinski  believes  that  genuine  pithiatic  symptoms  are  those  which 
can  be  produced  and  removed  by  suggestion.  Suggestion  thus  under- 
stood implies  the  idea  of  the  will  being  the  master  of  the  entire  situation. 
Babinski  does  not  believe  that  emotion  is  the  cause  of  hysteria.  It  is  a 
factor  totally  different  from  suggestion. 

Finally  he  removes  from  hysteria  also  exaggeration  of  tendon  reflexes 
and  vaso-motor  disturbances  of  the  skin. 

To  sum  up:  Babinski  considers  the  phenomena  of  hysteria  or  pithia- 
tisme depending  essentially  on  the  psychic  surroundings  or  on  the 
predisposition  of  the  patient.     They  are  the  result  of  suggestion  or  auto- 


480  HYSTERIA 

suggestion,  they  are  subordinate  to  the  will  of  the  patient.  Their  muta- 
bility is  characteristic. 

Babinski's  views  as  to  limitation  of  phenomena  belonging  to  hysteria 
are  too  radical.  While  it  is  true  that  simulation  (conscious  or  unconscious) 
is  met  with  in  a  number  of  cases  of  circulatory,  secretory  or  trophic  dis- 
turbances, nevertheless  observations  reported  by  competent  men  prove 
conclusively  that  hysteria  per  se  is  capable  to  produce  these  disturbances. 

Treatment. — It  was  mentioned  above  that  hysteria  develops  usually 
in  individuals  with  a  morbid  hereditary  history.  A  tendency  to  hysteria 
or  to  other  nervous  affections  is  therefore  great.  Consequently  prophy- 
laxy should  play  a  considerable  role  in  treatment  of  hysteria.  Nothing 
should  be  neglected  in  building  up  the  body.  General  hygiene,  hydro- 
therapy (cold  or  warm  water — according  to  the  tolerance),  massage, 
moderate  outdoor  exercise,  good  nutritious  food  are  the  general  measures. 

As  these  individuals  are  very  impressionable,  special  care  must  be 
taken  in  selecting  occupations.  The  latter  must  be  free  from  emotional 
incidents  as  far  as  possible.  The  entire  life  of  hystericals  must  be  so 
regulated  as  to  avoid  shocks,  emotions  of  any  nature.  As  they  show  a 
tendency  to  suggestion  and  autosuggestion,  they  must  avoid  emotional 
and  pathetic  scenes,  and  read  only  books  free  from  fantastic  and  terrifying 
descriptions. 

The  meals,  sleep  and  healthful  recreation  must  be  regulated  and 
strictly  adhered  to.  Stimulants,  including  tea  and  coffee,  are  forbidden. 
Smoking  should  be  avoided  as  much  as  possible.  Sexual  intercourse  must 
be  extremely  moderate.  The  summer  months  should  be  spent  in  a  quiet 
place,  free  from  undue  excitement,  although  some  pleasurable  entertain- 
ment is  permissible.  It  is  also  advisable  to  take  short  vacations  during 
the  working  months  of  the  year.  By  all  these  preventive  measures, 
applied  at  an  early  age  of  life,  the  patient  will  grow  up  physically  and 
mentally  strong  and  his  resistant  power  to  shocks  or  violent  emotions 
will  be  thus  greatly  increased. 

The  treatment  of  hysterical  manifestations  is  largely  psychic.  In 
presence  of  localized  palsies,  contractures,  anaesthesias,  globus  hystericus, 
aphonia,  paroxysms,  etc.,  all  symptoms  of  psychic  nature,  the  physician 
must  endeavor  to  convince  the  patient  that  the  trouble  is  not  serious,  is 
curable  and  that  by  strict  adherence  to  the  rules  of  the  treatment  he  will 
make  a  complete  recovery.  This  suggestion  and  persuasion  (psycho- 
therapy) must  be  repeated  frequently  and  its  manner  must  be  modified 
according  to  the  variations  of  the  patient's  condition.  It  must  be  done 
carefully  and  tactfully.  By  doing  so  the  patient  will  gain  confidence  and 
a  new  orientation  of  the  mental  processes  will  thus  be  brought  about  with 


HYSTERIA  481 

the  greatest  facility.  All  that  was  said  of  the  psychotherapeutic 
procedures  in  the  chapter  on  Psychasthenia  is  applicable  to  Hysteria.  As 
to  Psychoanalysis,  it  was  mentioned  that  Freud  considers  Hysteria  as  a 
psychoneurosis  and  therefore  the  psychoanalytical  method  finds  its 
proper  place  in  treatment  of  Hysteria. 

It  is  perfectly  legitimate  to  employ  some  physical  means  in  order  to 
reinforce  the  ordinary  suggestion.  For  example,  a  mild  electrical  current 
applied  to  the  neck  in  case  of  aphonia  or  globus  hystericus,  to  the  muscles 
of  a  contractured  segment  of  a  limb  may  give  excellent  results.  In  such 
cases  the  electricity  does  not  possess  a  special  curvative  power,  but  acts 
as  a  suggestive  agent. 

Simple  manipulations  of  the  affected  region  when  administered  regu- 
larly, so  as  to  impress  the  patient  as  if  it  were  a  necessary  procedure,  may 
yield  some  results.  Hysterical  backache,  headache,  neuralgia  of  long 
standing  have  been  cured  by  application  of  liniments,  water-bags,  etc. 

Hypnosis,  which  at  a  certain  period  reigned  in  treatment  of  functional 
nervous  diseases,  should  never  be  employed.  Hypnotic  sleep  exaggerates 
the  suggestibility  of  the  individual  and  practically  induces  hysteria  and 
consequently  aggravates  the  preexisting  hysterical  state.  It  should  be 
totally  abandoned.  When  the  hysterical  phenomena  are  marked,  isola- 
tion should  be  practised  in  conjunction  with  suggestive  treatment. 

Removal  of  the  patients  from  their  usual  surroundings  has  given  me 
excellent  results.  The  patients  gradually  get  accustomed  to  their  new 
life,  become  obedient  and  fall  entirely  under  the  physician's  control. 
This  method  gives  the  patient  a  simple,  quiet  and  regular  life;  all  sources 
of  irritation  are  thus  removed.  In  cases  of  marked  emotionality,  restless- 
ness, tremor  and  spasms,  rest  in  bed  is  very  beneficial.  Massage,  hydro- 
therapy will  then  be  excellent  adjuvants  in  preventing  wasting  and 
sluggish  digestive  function  (Weir-Mitchell's  plan). 

Medications  will  be  administered  only  in  cases  of  special  indications. 
Restlessness,  undue  emotionality  (crying,  laughing,  etc.)  can  be  combated 
by  bromides  and  tepid  baths.  For  insomnia  small  doses  of  veronal  or 
bromides  can  be  given.  When  the  patient  gets  accustomed  to  the  drug 
and  expects  his  capsule  at  night,  veronal  should  be  substituted  by  some 
placebo,  as  bicarbonate  of  soda,  or  plain  flour.  Gastro-intestinal  dis- 
turbances should  be  treated  accordingly.  A  hysterical  paroxysm  may  be 
prevented  by  sprinkling  cold  water  on  the  face  or  by  inhalation  of  a  few 
drops  of  ether.  In  one  of  my  female  patients  I  succeeded  in  checking  a 
paroxysm  by  an  application  of  a  faradic  current  to  the  left  hand.  In 
another  of  my  male  cases  the  patient  regained  consciousness  after  a  slight 
pressure  was  exercised  upon  the  left  hypochondrium. 
31 


482  EPILEPSY 

Speaking  generally,  very  little  medication  is  necessary  in  treatment 
of  hysteria. 

To  sum  up,  rest,  tonifying  measures,  isolation,  suggestion  in  a  waking 
state  (not  hypnosis),  persistent  efforts  to  remove  fixed  ideas,  which  are 
so  characteristic  of  the  disease  by  persuasion  or  psychoanalysis — this  is 
the  main  treatment  of  hysteria. 

EPILEPSY 

Epilepsy  is  characterized  by  a  sudden  loss  of  consciousness  with  (or 
without)  convulsions. 

Forms. — •   I.  Focal  or  Jacksonian  Epilepsy. 
II.  Major  Epilepsy  (Grand  Mai). 

III.  Minor  Epilepsy  (Petit  Mai). 

IV.  Equivalents  of  Epileptic  Attacks. 

I.  Focal  or  Jacksonian  Epilepsy 

See  description  in  Diseases  of  the  Brain. 

II.  Major  Epilepsy  (Grand  Mai) 

Essential  or  Idiopathic  Epilepsy. 

Symptoms. — The  disease  is  essentially  paroxysmal. 

A  seizure  is  usually  preceded  by  prodromal  symptoms  or  aurae  (warn- 
ings). The  latter  may  be  of  long  or  short  duration,  may  be  of  motor, 
sensory,  psychic,  vaso-motor  or  visceral  nature. 

A  motor  aura  consists  of  tremor,  of  twitching  affecting  one  or  several 
muscular  groups,  vertigo,  of  automatic  movements,  as  running,  striking, 
etc. 

A  sensory  aura  may  present  itself  as  an  epigastric  pain,  headache, 
sensation  of  cold  or  heat,  of  numbness,  of  tingling  (usually  in  the  limb  in 
which  the  convulsion  will  commence),  sensation  of  a  vapor.  Olfactory  and 
gustatory,  visual  and  auditory  disturbances  may  also  constitute  an  aura. 
The  "dreamy  state"  described  by  H.  Jackson  as  uncinate  fits  is  accom- 
panied by  a  smacking  of  the  lips  which  suggests  a  gustatory  phenomenon. 
"Sudden  darkness"  is  a  frequent  visual  aura,  special  colors,  images  may 
also  appear.  Sounds  of  a  whistle,  bell  or  of  a  voice — are  the  auditory 
aurae. 

A  psychic  aura  is  particularly  interesting,  as  it  shows  the  role  of  the 
brain  in  the  causation  of  epileptic  seizures.  It  may  consist  of  a  delirious 
state,  of  a  depression  or  else  of  gaiety,  of  terror,  of  sudden  appearance 
of  old  images,  etc. 


EPILEPSY  483 

Vasomotor  aura  presents  itself  as  a  sudden  pallor  or  else  redness  of 
the  face,  perspiration. 

Visceral  aurae  are:  cardiac  pain,  palpitation,  dyspnoea,  laryngeal  spasm, 
nausea,  vomiting,  cramps.  Epigastric  aura  is  one  of  the  most  common. 
It  may  be  a  pain  associated  with  nausea,  which  rapidly  ascends  to  the 
throat  or  to  the  head  and  is  followed  by  loss  of  consciousness. 

Cephalic  aura  consists  of  a  sensation  of  "fulness"  or  "rush  of  blood" 
in  the  head.  It  is  accompanied  by  giddiness.  Aurse  do  not  always  pre- 
cede an  attack.  The  latter  may  come  on  suddenly  without  the  least  pre- 
monitory symptoms.  In  exceptional  cases  an  attack  may  be  prevented 
or  checked  at  the  very  onset  by  certain  manipulations  as,  for  example, 
by  tightly  constricting  the  wrist.  In  such  cases  the  patient  carries  a 
small  girdle  on  the  wrist  with  a  string  attached  to  it.  As  soon  as  an  aura 
appears,  the  patient  immediately  pulls  at  it;  constriction  is  then  produced. 
One  of  my  patients  could  inhibit  an  attack  by  putting  some  food  in  his 
mouth  as  soon  as  his  aura  (pain  in  the  epigastrium)  appeared.  It  may  be 
arrested  also  by  strong  sensory  impressions,  such  as  smelling  strong  odors, 
or  a  splash  of  cold  water  on  the  face.  Sometimes  inhalation  of  amyl- 
nitrite  is  sufficient. 

The  seizure  itself  is  characterized  by  the  following  symptoms. 

The  patient  becomes  pale,  screams  and  falls  unconscious.  Immedi- 
ately tonic  convulsions  appear.  The  entire  body  becomes  rigid.  The 
head  is  turned  backward  or  to  one  side.  The  arms  and  legs  are  extended, 
the  hands  are  closed  and  the  thumbs  adducted.  The  trunk  is  immobile. 
The  eyeballs  are  bulging  and  turned,  the  pupils  dilated.  The  face,  which 
was  pale  at  the  beginning,  becomes  bluish  and  bloated.  The  tongue  if 
protruded  between  the  tight  jaws  bleeds.  Involuntary  evacuation  of 
urine  frequently  occurs,  probably  because  of  a  convulsive  spasm  of  the 
walls  of  the  bladder.  The  disturbance  of  circulation  (venous  stasis)  pro- 
duces sometimes  ecchymoses  and  epistaxis.  The  duration  of  the  tetanic 
phase  is  about  hah  a  minute. 

The  following  phase,  which  lasts  from  a  half  to  several  minutes,  is 
characterized  by  clonic  convulsions.  Here  the  rigidity  is  replaced  by 
convulsive  seizures.  The  latter  rapidly  follow  each  other,  are  abrupt  and 
irregular.  The  muscles  of  the  entire  body  twitch.  The  head  turns  from 
side  to  side,  the  grimaces  of  the  face  are  frightful.  The  cyanosis  disappears 
as  the  breathing  becomes  less  difficult.  Saliva  froths  at  the  mouth.  The 
alternative  closure  and  opening  of  the  jaws  cause  the  tongue  to  be  bitten. 
The  eyeballs  turn  rapidly  in  all  directions.  The  body  is  covered  with 
perspiration.  The  muscular  contractions  of  the  limbs  may  be  so  violent 
that  dislocations  occur.     Gradually  the  twitchings  subside  and  the  patient, 


484  EPILEPSY 

exhausted,  enters  the  third  stage  of  the  paroxysms.  In  the  majority  of 
cases  he  falls  asleep.  The  sleep  is  profound  and  may  last  several  h|ours. 
Awakened  he  has  no  recollection  of  his  attack.  Sometimes  the  amnesia 
covers  a  period  of  several  hours  preceding  the  attack  (retrograde  amnesia). 
Sleep  does  not  always  terminate  an  attack.  The  patient  then  opens  his 
eyes,  is  confused  and  remains  so  for  some  time.  In  exceptional  cases  the 
patient  in  sleep  turns  on  his  face  and  as  the  sleep  is  very  deep,  asphyxia 
may  occur. 

Certain  objective  phenomena  usually  accompany  or  follow  an  epi- 
leptic seizure. 

The  temperature  rises  during  an  attack,  but  not  above  0.50.  The 
pupils  are  dilated  during  the  tetanic  phase,  but  myotic  in  the  subsequent 
phase.  The  pupillary  reflex  is  abolished.  The  tendon  and  cutaneous 
reflexes  are  exaggerated.  I  have  observed  frequently  increased  knee-jerks, 
also  sometimes  ankle-clonus  and  Babinski  sign,  but  paradoxical  reflex  is 
met  more  frequently  than  the  latter.  This  state  of  tendon  reaction 
is  continued  for  some  time  after  the  attack.  Relaxation  of  anal 
sphincter  is  frequent  during  a  seizure. 

Paralytic  phenomena  sometimes  follow  an  attack.  They  are  the  result 
of  cerebral  exhaustion.  Hemiplegia,  monoplegia,  asphasia  may  occur, 
but  they  are  usually  of  a  transient  nature.  Concentric  contraction  of 
the  visual  field  and  ocular  palsies  are  occasionally  observed.  The  general 
nutrition  usually  suffers,  especially  when  the  attacks  are  frequent.  Phos- 
phaturia  and  albuminuria  are  not  infrequent.  The  toxicity  of  urine  is 
diminished  before  and  during  the  attack,  but  increased  after  an  attack. 
The  toxicity  of  the  blood  serum  is  increased  during  an  attack  and  the 
toxicity  of  the  cerebro-spinal  fluid  is  increased  after  an  attack. 

The  mental  depression  following  an  epileptic  seizure  presents  varia- 
tions from  a  slight  hebetude  to  stupor.  It  increases  in  intensity  when  the 
attacks  are  frequent.  In  the  latter  case  a  feebleness  of  the  mental  faculties 
develops  gradually  and  if  the  attacks  are  not  kept  under  control,  a  true 
and  permanent  dementia  will  ensue.  In  children  frequently  repeated 
epileptic  seizures  will  gravely  interfere  with  the  intellectual  development 
and  mental  arrest  will  be  the  result. 

Status  Epilepticus. — Under  this  term  is  understood  a  series  of  attacks 
which  rapidly  follow  each  other  without  the  patient  regaining  conscious- 
ness. This  form  of  epilepsy  is  very  grave.  Death  may  occur  during 
the  convulsive  period  or  during  the  stage  of  stupor.  However  the 
condition  is  not  invariably  fatal.  When  the  intervals  between  indi- 
vidual series  of  attacks  increase,  the  patient  may  regain  consciousness 
and  improve. 


EPILEPSY  485 

Atypical  Epileptic  Seizures. — An  epileptic  attack  as  described  above 
does  not  always  present  itself  in  its  typical  form.  It  varies  in  different 
individuals  and  even  in  the  same  individual.  The  phase  of  tonic  convul- 
sions, for  example,  may  be  extremely  brief  and  this  phase  may  represent 
the  entire  attack.  In  other  cases  the  clonic  phase  may  be  unusually 
prolonged  even  as  long  as  15  minutes.  The  clonic  contractions  may  be 
of  wider  range  than  in  typical  epilepsy  and  they  may  give  the  impression 
of  a  hysterical  paroxysm;  they  are  therefore  called  "hysteroid."  They 
usually  alternate  with  typical  epileptic  convulsions,  but  when  they  do 
not,  the  diagnosis  between  hysteria  and  epilepsy  is  very  difficult  and 
remains  necessarily  in  suspense  until  a  classical  attack  occurs. 

There  are  cases  in  which  clonic  convulsions  may  not  affect  the  entire 
body.  When  the  tonic  phase  alone  exists,  the  convulsive  movements 
pass  into  muscular  relaxation  without  the  clonic  spasms.  It  is  a  serious 
condition :  symptoms  of  asphyxia  are  marked  toward  the  end  of  the  attack. 
The  rigidity  of  various  muscles  is  quite  pronounced:  tetanic  contraction 
of  the  jaws  and  opisthotonos  may  be  observed.  In  some  rare  cases  the 
attack  may  consist  only  of  a  sudden  loss  of  consciousness  with  falling  but 
without  convulsions.  This  is  the  so-called  apoplectic  form  of  epilepsy. 
Usually  such  attacks  alternate  with  convulsive  attacks. 

In  spite  of  the  fact  that  loss  of  consciousness  and  inability  to  recall 
the  attack  are  characteristic  of  epilepsy,  nevertheless  there  are  caess1  in 
which  the  individual  is  conscious  of  what  is  going  on  during  an  attack 
but  forgets  after  the  attack  is  over,  also  are  there  cases  in  which  con- 
sciousness is  preserved  as  well  as  memory  after  the  attack.  I  have  seen 
cases  in  which  patients  witnessed  the  attacks.  Such  cases  may  be  taken 
for  hysteria  if  they  do  not  alternate  with  typical  seizures.  They  are 
called  hysteroid.  There  are  other  atypical  occurrences  among  varieties 
of  epilepsy.  The  initial  cry  and  the  biting  of  the  tongue  may  be  absent. 
In  some  cases  the  seizures  occur  only  at  night  and  therefore  are  not 
recognized  until  an  occasional  bleeding  of  the  tongue  or  an  involuntary 
micturition  attracts  attention. 

The  deviations  from  the  regular  type  of  epilepsy  may  be  multiple,  but 
what  characterizes  all  the  typical  varieties  of  epilepsy  is  the  total  inability 
of  the  patient  to  realize  what  occurred  during  an  attack  when  conscious- 
ness is  regained. 

m.  Minor  Epilepsy  (Petit  Mai) 

It  is  characterized  by  a  sudden  loss  of  consciousness  lasting  but  a  few 
seconds.     In  the  midst  of  a  conversation,  of  reading,  of  playing  an  in- 


486  EPILEPSY 

strument,  of  eating,  etc.,  the  patient  suddenly  becomes  pale,  interrupts 
the  act  he  is  doing  and  loses  consciousness.  In  a  few  seconds  the  attack 
is  over  and  he  resumes  his  work.  Like  in  major  epilepsy  there  is  total 
amnesia  of  the  attack  after  consciousness  is  regained.  The  attack  is  usually 
so  brief  that  the  patient  does  not  fall.  In  a  great  many  cases  some  sen- 
sation precedes  the  attack,  such  as  sudden  light  or  appearance  of  colors. 

Petit  mal  may  present  itself  in  various  forms.  In  some  patients  an 
attack  is  noticed  from  sudden  staring  of  the  eyes.  Others  are  taken  with 
a  sudden  vertigo  of  a  very  short  duration.  One  of  my  patients  would  be 
taken  several  times  a  day  with  a  sudden  inability  to  realize  where  he  was. 

IV.  Epileptic  Equivalents 

They  consist  of  motor,  sensory,  visceral  and  psychic  manifestations 
which  are  sudden  in  onset  and  accompanied  by  total  loss  of  consciousness. 

To  this  group  belongs  the  so-called  ambulatory  form  of  epilepsy.  The 
patient  thus  affected  usually  without  and  exceptionally  with  a  few  pre- 
monitory symptoms,  as  headache,  depression,  etc.,  suddenly  leaves  his 
home  and  automatically  wanders  away;  sometimes  he  gets  on  the  train 
and  travels  hours  or  days.  Suddenly  he  realizes  his  situation  and  is 
astonished  to  find  himself  removed  from  his  home.  Similarly  to  genuine 
epilepsy  there  is  total  amnesia  of  the  accomplished  act.  In  another  form 
of  the  disease  there  is  an  irresistible  desire  for  sleep.  It  comes  on 
suddenly  and  in  attacks.     It  may  terminate  with  a  mild  delirium. 

Equivalents  of  epilepsy  are  also  seen  in  a  sudden  irresistible  desire  to 
do  unusual  acts.  One  of  my  patients  had  attacks  in  which  he  would 
suddenly  undress  himself.  Another,  a  clerk,  would  suddenly  unbutton 
his  trousers  in  his  office  and  urinate. 

Various  subjective  sensations  or  hallucinations,  visceral  disturbances, 
as  pain  in  the  abdomen,  sudden  desire, for  defecation,  attacks  of  angina 
pectoris,  of  migraine,  of  syncope,  of  asthma,  may  be  considered  as  equiva- 
lents of  epilepsy  when  their  onset  is  sudden,  their  duration  is  short  and 
when  there  are  no  organic  visceral  changes  that  could  account  for  the 
disturbance. 

The  so-called  psychic  epilepsy  belongs  to  the  same  group.  The  pa- 
tient thus  affected  is  suddenly  taken  with  a  desire  to  do  the  most  absurd 
and  impossible  acts.  Sometimes  he  attacks,  injures  or  kills.  Not  in- 
frequently he  is  prompted  in  his  actions  by  various  auditory  or  visual 
hallucinations.  The  seizure  terminates  as  suddenly  as  it  began.  Some- 
times it  is  followed  by  a  state  of  exhaustion  or  depression.  In  some  cases 
the  onset  of  the  attack  is  preceded  by  headache,  undue  irritability,  depres- 


EPILEPSY  487 

sion.  The  characteristic  feature  of  the  seizure  is  the  complete  in- 
ability of  the  patient  to  recall  a  single  act  committed  by  him  during  the 
attack. 

Pavor  Nocturnus  or  Night  Terrors.  — In  adults  the  condition  occurs 
in  the  beginning  of  sleep.  Suddenly  the  body  is  seized  with  a  very  pro- 
nounced jerk  and  the  patient  at  once  sits  up.  He  wakes  up,  is  frightened 
and  the  heart  beats  rapidly.  Considering  the  fact  that  individuals  suffer- 
ing from  this  manifestation  are  usually  epileptics,  the  relation  of  it  to 
epilepsy  becomes  evident. 

In  children  the  same  sudden  onset  with  the  state  of  anxiety  is  present 
but  in  addition  to  it  there  are  also  frightful  hallucinations  with  amnesia. 
The  latter  manifestations  are  assuredly  analogous  to  some  equivalents 
of  epilepsy  mentioned  above.  There  are  cases  on  record  pointing  to  the 
fact  that  night  terrors  are  in  reality  attacks  of  petit  mal,  also  that  they 
eventually  are  substituted  by  genuine  attacks  of  epilepsy. 

Epileptic  Character. — Epileptic  individuals  very  frequently  present, 
certain  peculiarities  of  character  which  deserve  to  be  mentioned.  Irrit- 
ability, quite  pronounced,  is  characteristic.  Anger  is  brought  out  upon 
the  least  provocation.  With  it  maliciousness  is  frequently  exhibited 
even  toward  his  own  people.  Obstinacy  is  another  feature;  it  is  quite 
pronounced.  It  frequently  accompanies  a  morbid  egotism.  This  leads 
to  excessive  impulsiveness  in  actions  and  words ;  it  is  exhibited  even  in  the 
most  insignificant  occurrences.  The  obstinacy  naturally  shows  deficient 
judgment  and  illogicality.  Moral  sentiments  are  also  at  fault.  The 
morbid  egotism  mentioned  above  develops  an  unforgiving  spirit  toward 
those  who  happened  to  injure  them  even  slightly.  A  certain  revenge- 
fulness  and  even  cruelty  are  not  infrequently  noticed  in  the  epileptics  in 
their  relation  to  others. 

Course,  Termination,  Prognosis. — There  are  great  variations  in  the 
course  and  duration  of  epilepsy.  Some  patients  may  have  several  seizures 
daily,  others  only  one  attack  in  weeks  or  months;  some  only  during  the 
night,  others  during  the  day.  Petit  mal  attacks  occur  only  during  waking 
hours.  Some  epileptics  have  only  attacks  of  grand  mal,  others  only  of 
petit  mal  and  still  others  have  both  forms.  Intervening  diseases  some- 
times arrest  the  seizures.  Gastro-intestinal  disturbances  have  a  consider- 
able influence  upon  the  frequency  of  the  attacks.  Constipation  is  fre- 
quently followed  by  seizures.  Use  of  stimulants  and  excesses  of  any  kind 
increase  the  intensity  of  individual  attacks  as  well  as  their  frequency. 

Menstruation  may  have  some  relation  to  attacks  of  epilepsy.  In  a 
certain  group  of  cases  the  seizures  occur  only  around  the  time  of  men- 
struating periods.     Perhaps  the  disturbance  in  the  internal  secretion  of  the 


488  EPILEPSY 

ovaries  plays  a  certain  direct  or  indirect  role  on  the  cerebral  irritation 
thus  producing  convulsions. 

Oncoming  puberty,  which  ordinarily  is  associated  with  physiological 
disturbances  in  the  organism,  usually  increases  the  severity  or  frequency 
of  epileptic  seizures.  Sometimes  epilepsy  makes  its  first  appearance  at 
puberty. 

Not  infrequently  the  disease  begins  with  attacks  of  petit  mal  which 
gradually  may  pass  into  major  seizures.     Sometimes  both  forms  coexist. 

When  the  disease  commences  early  in  infancy,  it  has  a  slight  tendency 
to  disappear  at  the  age  of  ten.  The  same  tendency  is  also  observed  in 
middle  life. 

Life  is  not  directly  threatened  by  epilepsy,  except  when  injuries  occur 
in  falling.  Status  epilepticus  is  very  dangerous  to  life.  In  the  majority 
of  cases  epilepsy  is  not  curable.  Recoveries  follow  very  occasionally. 
The  disease  may  last  many  years.  Petit  mal  is  more  obstinate  and  less 
easily  influenced  by  treatment  than  grand  mal.  Mental  failure  frequently 
accompanies  the  advanced  cases  of  epilepsy  and  in  such  cases  dementia 
is  the  ultimate  outcome.  Epilepsy  beginning  in  early  life  interferes  with 
the  mental  development  and  imbecility  is  frequent. 

Alcoholic  and  syphilitic  epilepsy  are  sometimes  amenable  to  treatment 
and  present  a  favorable  prognosis. 

Diagnosis. — In  the  majority  of  cases  the  individual  phenomena  of  an 
epileptic  seizure  are  so  typical  that  there  is  no  difficulty  in  making  a  diag- 
nosis. In  the  atypical  or  irregular  forms  (see  above)  the  symptoms  may 
simulate  other  affections. 

A  hysterical  paroxysm  is  recognized  by  the  following  symptoms.  It 
never  occurs  at  night  during  sleep.  Its  onset  is  not  sudden  and  not  accom- 
panied by  an  initial  cry.  There  is  no  biting  of  the  tongue  and  no  involun- 
tary loss  of  urine  or  feces.  The  muscular  twitchings  are  of  wide  range, 
and  they  possess  a  "purposive"  character.  The  pupillary  reflexes  are 
intact.  The  attack  does  not  terminate  by  stupor  or  coma,  but  by  an 
outbreak  of  laughing  or  crying.  If  sleep  occurs,  it  is  superficial  and  not 
as  profound  as  in  epilepsy.  Mental  failure  or  dementia,  which  is  observed 
in  epilepsy  of  long  standing,  is  not  present  in  hysteria.  Hysterical 
attacks  are  amenable  to  treatment  sometimes  by  suggestion. 

Uremic  convulsion  occurs  without  an  initial  cry  or  without  an  aura. 
There  is  no  biting  of  the  tongue.  The  urinary  examination  will  show 
quantitative  and  qualitative  changes  characteristic  of  diseases  of  the 
kidneys. 

Epileptiform  convulsions  may  occur  in  cases  with  increased  intra- 
cranial pressure,  as  tumors,  meningitis,  etc.     They  may  also  occur  in 


EPILEPSY  489 

the  course  of  paresis.  It  is  therefore  important  to  eliminate  organic 
diseases  in  every  case  presenting  a  history  of  convulsions. 

Attacks  of  petit  mal  present  no  diagnostic  difficulty  from  their  sudden 
onset,  sudden  termination,  extremely  brief  and  absolute  loss  of  con- 
sciousness. 

The  psychic  form  of  epilepsy  presents  at  times  great  difficulties,  as 
irresistible  impulses  are  observed  in  the  course  of  psychoses  accompanied 
by  delusions  and  hallucinations.     The  diagnosis  will  be  made  by  exclusion. 

The  equivalents  of  epilepsy  (see  above),  as  attacks  of  vertigo,  syncope, 
angina  pectoris,  etc.,  will  be  easily  recognized  by  eliminating  visceral 
diseases  (labyrinth,  heart,  etc.).  In  aural  vertigo  there  is  no  loss  of 
consciousness.  In  syncope  which  usually  follows  an  emotion  or  occurs  in 
an  overheated  room  loss  of  consciousness  takes  place,  but  when  the  latter 
is  regained,  it  is  perfect  from  the  beginning.  The  same  is  not  observed  in 
epilepsy.  Gowers  believes  that  repeated  attacks  of  syncope  which  are 
due  to  cardiovascular  changes  with  alteration  in  the  cerebral  circulation 
produce  a  lessened  resistance  of  the  nervous  mechanism  of  the  cardio- 
vascular apparatus,  the  reflex  becomes  easier  and  loss  of  consciousness 
more  sudden.  He  believes  that  a  change  in  nerve  elements  of  the  brain 
occurs  in  syncope  and  by  virtue  of  frequent  repetition  of  the  attacks,  the 
state  of  nerve  elements  acquires  a  tendency  to  spontaneous  development 
which  is  characteristic  of  petit  mal.  Thus  an  ordinary  faint  or  syncope 
may  eventually  develop  into  minor  epilepsy. 

Pathogenesis  of  Epilepsy. — Post-mortem  examinations  have  given 
nothing  definite  as  to  a  possible  organic  cause  of  essential  epilepsy.  In 
spite  of  this  fact  experimental  investigations  (Fritch,  Hitzig,  Charcot, 
Francois-Frank  and  others)  have  shown  that  convulsions  are  essentially 
a  cortical  phenomenon.  An  irritation  of  any  area  of  the  cerebral  cortex, 
but  particularly  of  the  motor  area,  produces  convulsive  seizures.  Francois- 
Frank  has  also  shown  that  cortical  irritation  is  followed  by  vascular  spasm, 
by  changes  in  cardiac  rhythm,  by  dilatation  of  pupils,  by  incontinence  of 
urine  and  feces;  otherwise  speaking,  by  the  symptom-group  observed 
during  an  epileptic  seizure. 

It  is  now  generally  conceded  that  the  point  of  departure  of  a  con- 
vulsive attack  or  of  petit  mal  attack  lies  in  the  cortex,  which  at  that 
moment  undergoes  an  undue  irritation.  Various  factors  are  supposed 
to  cause  this  irritation.  Anaemia,  hyperemia  of  the  brain,  toxic  sub- 
stances originating  in  the  organism  (autointoxication)  or  introduced  from 
without  (alcohol,  etc.)  are  considered  the  immediate  exciting  factors 
capable  of  producing  a  cortical  discharge  in  the  form  of  grand  mal,  petit 
mal  or  equivalent  of  epilepsy. 


490  '  EPILEPSY 

In  favor  of  the  cortical  origin  of  epilepsy  speaks  also  the  fact  that 
mental  feebleness  and  dementia  develop  in  the  course  of  the  disease. 
The  toxic  nature  of  essential  epilepsy  is  corroborated  by  the  influence  of 
gastro-intestinal  disturbances  upon  the  intensity  and  frequency  of  its 
manifestations,  by  the  degree  of  toxicity  of  the  urine  and  of  the  cerebro- 
spinal fluid  before  and  after  the  attacks.  Not  infrequently  at  autopsy  of 
epileptics  old  meningeal  or  cortico-meningeal  lesions  are  found.  Such  in- 
dividuals may  have  had  attacks  in  their  younger  years  which  gradually  dis- 
disappeared  or  else  occurred  since  at  very  rare  intervals.  Recurrences  in 
such  cases  take  place  a.  propos  of  an  autointoxication  (gastro-intestinal, 
renal,  glandular).  Experiments  on  animals  show  that  an  intoxication 
which  is  without  an  effect  on  a  normal  animal  is  a  cause  of  epileptic 
seizures  in  an  animal  presenting  old  cortico-meningeal  lesions  (Claude 
and  Lejonne,  Comptes-rendus  de  Soc.  de  Biologie,  1910).  Guidi  (Rivista 
sperim.  di  Friniatria,  1908),  has  also  shown  experimentally  that  there 
are  profound  metabolic  changes  in  epileptics:  transformation  of  proteids 
is  not  like  in  normal  individuals;  the  most  manifest  deviation  is  seen  in  the 
production  of  urea,  hence  an  acid  intoxication  takes  place;  excess  of 
ammoniacal  compounds  is  found  in  the  urine  of  epileptics.  Removal  of 
these  chemical  compounds  by  an  appropriate  diet  reduces  the  number 
and  intensity  of  seizures. 

Etiology. — The  causes  of  epilepsy  are  predisposing  and  exciting.  A 
neuropathic  heredity  (epilepsy,  other  neuroses,  insanity  in  the  family), 
consanguinity,  hereditary  alcoholism,  syphilis,  lead  poisoning,  are  all 
predisposing  factors.  The  influence  of  heredity  is  considerable.  The 
exciting  causes  are :  infections,  intoxications,  traumata.  Among  infectious 
diseases  scarlet  fever,  typhoid  fever,  measles  and  smallpox  play  a  con- 
siderable role.  Gastro-intestinal  disturbances  are  a  common  cause  for 
epileptic  convulsions.  Alcohol  taken  in  large  doses  and  for  a  prolonged 
period  of  time  is  a  potent  factor  in  epilepsy.  It  is  possible,  however,  that 
arterial  degeneration  with  the  rise  of  blood  pressure  are  apt  to  produce 
cortical  irritation  and  therefore  epileptiform  attacks  in  aged  individuals 
(Senile  Epilepsy).  Among  other  toxic  elements  capable  of  causing 
epilepsy  can  be  mentioned:  lead,  mercury,  cocain,  morphin,  chloroform, 
ether. 

Syphilis  may  produce  epilepsy  without  definite  anatomical  lesions, 
especially  during  the  secondary  period. 

Gout,  diabetes,  ansemia,  cardiac  diseases  and  circulatory  disturbances 
in  general  are  also  considered  as  causes  of  epilepsy,  but  their  relation  to 
the  disease  is  not  entirely  elucidated.  Alcohol  may  be  an  exciting  cause 
for  attacks  in  an  epileptic  individual  or  per  se  be  a  factor  in  development  of 


EPILEPSY  491 

epilepsy  in  a  neuropathic  individual.  In  chronic  alcoholic  persons  who 
used  alcohol  to  excess  for  a  very  prolonged  period,  withdrawal  of  alco- 
hol is  likely  to  bring  on  epileptic  seizures.  This  is  observed  particularly 
in  asylums.  Traumata  may  cause  epilepsy  either  through  direct  injury 
of  cerebral  tissue  or  only  in  cases  of  concussion  without  apparent  lesions. 
In  considering  trauma  as  a  cause  it  should  be  borne  in  mind  that  the 
symptoms  of  epilepsy  may  appear  shortly  after  the  accident  or  a  long 
time  later,  as  late  as  ten,  fifteen  or  twenty  years.  Peripheral  injuries, 
viz.  of  an  eye,  ear,  nasal  fossae,  of  a  nerve,  may  be  the  direct  cause  of 
convulsive  seizures. 

The  relation  of  epilepsy  to  visceral  diseases,  as  uterus,  intestinal 
worms,  polyps  in  the  nose  or  ear,  errors  of  refraction,  etc.,  is  maintained 
by  some  observers,  but  not  definitely  proven.  These  cases  are  the  so- 
called  "Reflex  Epilepsy."  A  large  number  of  cases  date  from  infancy. 
Infantile  convulsions  or  petit  mal  occur  frequently  during  the  period  of 
dentition.  If  they  persist,  they  become  distinctly  epileptiform  at  puberty 
or  even  earlier.     Infantile  convulsions  may  cause  permanent  hemiplegia. 

Epilepsy  may  occur  at  any  age,  but  in  the  majority  of  cases  between 
the  age  of  ten  and  twenty.  There  are  cases  in  which  epilepsy  occurs 
only  during  or  immediately  before  the  menstrual  periods.  There  are 
also  cases  in  which  epilepsy  begins  only  at  puberty.  The  period  of  meno- 
pause has  sometimes  a  favorable  influence  on  epilepsy.  As  a  rule  the 
disease  is  rare  in  old  age,  but  senile  epilepsy  is  well  known. 

Treatment. — In  the  chapter  on  the  Pathogenesis  of  Epilepsy  cortical 
irritation  was  considered  as  the  underlying  immediate  cause  of  the  epileptic 
discharge.  The  main  indications  therefore  are:  (1)  Removal  of  factors 
producing  cortical  excitability  and  (2)  modification  of  the  latter  when  it 
exists. 

1 .  The  first  is  by  far  the  most  important.  An  effort  must  be  made  to 
place  the  patient  in  such  conditions  as  to  reduce  to  a  minimum  auto- 
intoxication. This  can  be  accomplished  by  an  appropriate  regime  and 
hygienic  rules.  It  has  been  my  experience  that  removal  of  meat  from  the 
diet  is  very  beneficial.  Starchy  food  and  sweets  should  be  avoided  or  at 
least  considerably  reduced  in  quantity.  The  patient  should  be  instructed 
to  have  his  meals  at  regular  hours  and  not  to  take  food  between  the  meals. 
The  latter  should  consist  of  milk,  soft-boiled  eggs,  vegetables,  fruit. 
These  articles  can  be  combined  in  such  a  manner  as  to  suit  the  patient's 
taste  and  afford  variations  in  the  meals.  Stimulants,  also  tea  and  coffee, 
should  be  avoided.  The  amount  of  food  for  each  meal  should  not  be 
abundant.  The  mastication  must  be  thorough  and  the  eating  should  be 
slow. 


492  EPILEPSY 

As  I  have  shown  with  other  observers  {New  York  Med.  Jour.,  1906), 
table  salt  is  to  be  avoided  entirely  or  taken  in  a  very  small  amount.  I 
have  found  that  there  is  a  decided  relation  between  the  intensity  and  the 
frequency  of  the  attacks  and  the  amount  of  table  salts  used  with  food. 
In  order  to  avoid  aversion  for  saltless  food  a  gradual  reduction  of  the 
amount  of  salt  is  recommended.  Experimentally  it  has  been  shown 
(Paderi,  Archivio  di  Farm,  sperim.  e  Scienze  affin.,  191 1)  that  the  effect  of 
bromides  on  the  organism  is  greater  and  more  rapid  when  the  diet  is 
salt-free. 

Proper  elimination  plays  a  prominent  role.  A  purgative  administered 
regularly  once  a  week  and  oftener,  if  there  is  a  tendency  to  constipation, 
is  a  necessity.  Special  emphasis  should  be  laid  on  regular  bowel  move- 
ment, as  there  is  almost  a  direct  relationship  between  constipation  and 
epileptic  attacks.  All  sources  of  autointoxication  must  be  removed. 
The  patient  is  also  instructed  to  keep  his  mouth  and  teeth  in  as  perfect 
condition  as  possible. 

The  mode  of  living,  occupation,  sleep,  etc.,  must  be  well  regulated. 
The  patient  must  go  to  bed  early  and  get  up  late,  so  as  to  get  a  great  deal 
of  rest.  An  hour's  sleep  during  the  day  is  also  desirable.  He  must  take 
two  or  three  walks  a  day,  each  of  such  duration  as  not  to  feel  fatigued. 
Fatigue  as  a  rule  must  be  avoided.  All  sporty  exercises  are  forbidden. 
The  occupation  must  be  of  such  a  nature  as  to  give  a  minimum  of  mental 
or  physical  strain.  Crowded  places,  as  theatre,  church,  etc.,  must  be 
avoided.  While  complete  seclusion  is  not  advisable  because  of  the  mental 
depression  it  leads  to,  entertainments,  society  gatherings,  games,  etc., 
should  be  avoided  as  much  as  possible.  Hydrotherapy  is  useful.  Shower 
baths,  warm  or,  better,  cold,  of  half  a  minute's  duration  and  followed  by 
a  gentle  general  massage — once  or  twice  a  day — are  very  beneficial.  A 
sojourn  in  the  country  several  times  during  the  year  is  advisable.  If  the 
patient  is  a  young  boy  or  girl,  they  shouid  be  kept  out  of  school.  Mental 
work  in  general  should  be  done  as  little  as  possible.  Sexual  intercourse 
should  be  avoided.  Marriage  is  not  to  be  advised,  first  because  of  the 
probability  of  sexual  excesses,  next  in  view  of  the  offspring  which,  as 
statistics  show,  in  the  majority  of  cases  inherit  a  neuroyathic  tendency. 
Imbeciles,  idiots  and  otherwise  mentally  deficient  have  come  from  parents 
suffering  from  epilepsy. 

This  is  the  regime  to  which  I  usually  submit  my  patients  suffering 
from  essential  epilepsy. 

Careful  examination  should  be  made  in  regard  to  local  irritation,  as 
polyps  of  the  nasal  cavities,  to  errors  of  refraction,  to  intestinal  parasites, 
to  scars,  to  injured  peripheral  nerves,  to  visceral  diseases.     Correction  or 


EPILEPSY  4Q3 

removal  of  these  factors  should  be  made  as  early  as  possible  in  the  course 
of  the  disease.  Incases  of  toxic  origin  rapid  elimination  of  toxic  products 
must  be  insisted  on.  In  cases  of  traumatic  epilepsy  a  thorough  search 
should  be  made  for  any  depression  of  the  cranium  or  for  a  localized 
pachymeningitis.  Surgical  intervention  is  of  course  necessary  in  such 
conditions.  In  traumatic  epilepsy  of  long  standing  operative  procedures 
should  be  undertaken.  An  effort  should  be  made  to  localize  the  lesion. 
A  Roentgen-ray  examination  should  be  of  considerable  assistance  in  such 
cases. 

In  absence  of  any  localizing  cause  on  the  cranium  or  at  the  periphery 
surgical  procedures  should  be  undertaken  in  idiopathic  epilepsy  with 
grea4  hesitation.  When  the  seizures  are  rare  and  slight,  also  when  medica- 
tions improve  the  condition,  no  operation  should  be  thought  of.  In  grave 
cases  which  are  rebellious  to  medical  treatment  and  which  by  virtue  of 
the  intensity  and  frequency  of  the  attacks  endanger  life,  surgical  inter- 
vention may  be  considered.  Trephining  and  osteoplastic  operations 
have  been  performed  but  without  permanent  success.  Recurrences  of 
attacks  is  the  rule,  although  amelioration  has  been  obtained  in  some 
cases.  In  one  of  my  cases  I  had  an  osteoplastic  operation  performed 
over  the  motor  area  of  one  side  and  six  months  later  over  the  other  side. 
Considerable  improvement  followed  not  only  with  regard  to  the  frequency 
of  the  seizures,  but  also  the  girl  (twelve  years  of  age)  began  to  show  con- 
siderable mental  progress. 

Operations  on  the  cervical  sympathetic  nerve  have  been  advised  by 
Chipault  and  Jonneseo,  but  the  results  so  far  obtained  do  not  apparently 
justify  their  use.  Giacomelli  (Gaz.  degli  Osped.  e  delle  Clin.,  1912) 
recommends  for  essential  epilepsy  intra-spinal  injections  of  stovain. 
After  withdrawing  by  lumbar  puncture  about  8  c.c.  of  cerebro-spinal  fluid 
he  injects  0.08  grm.  of  stovain  diluted  in  1  c.c.  of  physiological  solution 
of  chloride  of  sodium.  In  one  of  his  cases  there  was  complete  recovery, 
in  other  cases  a  considerable  reduction  of  the  number  of  seizures  and  of 
their  intensity  followed. 

2.  Cortical  excitability  will  as  a  rule  be  diminished  by  the  just  outlined 
method  of  general  management.  However  in  some  cases  the  latter  may 
not  be  sufficient.  Medications  are  very  frequently  necessary.  Bromides 
is  the  most  efficient  remedy  according  to  the  majority  of  observers  and 
among  all  the  salts  sodium  bromide  is  the  best  tolerated  by  the  patients. 
Its  doses  are  given  according  to  the  age.  I  have  had  better  results  in 
giving  small  doses  frequently  repeated  than  large  doses  once  or  twice  a 
day.  I  usually  prescribe  for  an  adult  at  the  beginning  gr.  x  every  three 
hours  and  at  the  end  of  three  or  four  days  every  two  hours.     If  the  attacks 


494  EPILEPSY 

are  not  controlled,  the  patient  is  told  to  take  it  every  hour.  When  an 
improvement  is  noticed  at  the  end  of  a  few  weeks,  the  same  amount  is 
given  only  every  four  hours.  An  excellent  adjuvant  to  the  bromides  is 
one  of  the  coal-tar  products,  and  I  always  combine  sodium  bromide  with 
antipyrin  in  gr.  v  doses.  This  treatment  must  be  kept  up  for  several 
months.  If  bromide  acne  appears,  the  drug  must  be  discontinued  for 
a  few  days  and  substituted  by  sulfonal  or  trional.  The  acne  can  be 
relieved  considerably  by  administration  of  arsenic  in  two,  three,  or  four 
drops  of  Fowler's  solution.  It  should  be  continued  as  long  as  bromides  are 
given.  In  the  majority  of  my  cases  I  persist  with  the  bromides  in  spite 
of  the  acne  and  no  special  ill  effects  have  I  noticed.  In  severe  cases  the 
gr.  x  dose  every  two  or  three  hours  may  not  be  sufficient.  Fifteen  or 
twenty  grains  may  then  be  given.  Bromides  sometimes  produce  a  marked 
general  depression  with  impairment  of  memory,  but  the  latter  are  only 
temporary  phenomena.  On  the  contrary,  in  a  number  of  instances  in 
my  experience  a  prolonged  administration  of  bromides  was  followed  by  a 
very  marked  lucidity  of  mind  because  the  drug  succeeded  in  removing  the 
seizures  for  long  intervals.  The  so-called  bromide  dementia  has  never 
been  observed  by  me. 

When  a  marked  intolerance  is  noticed  (which  is  exceptional)  bromides 
may  be  substituted  by  other  drugs,  as  extract  of  opium  in  doses  not  above 
fifteen  grains  a  day,  extract  of  solanum  carolinensis  in  doses  of  from 
fifteen  to  sixty  drops,  adonis  vernalis,  digitalis,  atropine,  belladonna, 
camphor,  valerian.  Any  of  these  drugs  can  be  given  conjointly  with  or 
in  place  of  bromides.  It  should  be,  however,  borne  in  mind  that  bromides 
are  the  most  efficacious  of  all  the  drugs  mentioned. 

When  a  syphilitic  history  is  present,  mercurials  and  iodides  are 
indicated. 

Iodides  are  also  useful  in  cases  with  a  history  of  lead  poisoning. 

There  is  a  certain  class  of  epileptics  which  I  found  can  be  benefited 
by  the  administration  of  thyroid  extract.  These  individuals  show  signs 
of  hypothyroidization.  I  made  a  special  study  of  them  (Therap.  Gazette, 
1907)  and  the  entire  series  of  my  patients  benefited  considerably  from 
this  medication.  The  epileptic  seizures  became  less  and  less  frequent 
and  the  mental  condition  improved  to  a  remarkable  degree. 

Leubuscher  {Deut.  Medi.  Wchn.  No.  n,  1913)  advocates  adminis- 
tration of  phosphorous  in  view  of  its  good  effect  in  tetany.  He  gives  it 
in  10,000  parts  oil.  In  his  cases  he  obtained  considerable  reduction  of 
the  number  of  seizures. 

The  treatment  of  epilepsy  must  be  kept  up  in  the  most  rigorous  and 
persistent  manner  and  efforts  be  made  to   control  the   attacks.     The 


CHOREA  495 

disease  itself  has  a  very  deleterious  effect  upon  the  mental  condition  of 
the  patient.  In  children  it  interferes  with  their  intellectual  growth. 
Mental  arrest  follows  if  the  intervals  between  the  seizures  are  not  length- 
ened. When  it  is  difficult  to  carry  out  the  above  outlined  treatment 
concerning  the  general  condition  of  the  patient  it  is  advisable  to  place 
him  in  a  special  institution  for  epileptics  (colonies  or  state  asylum). 

Treatment  during  an  Epileptic  Seizure.— In  the  chapter  on  sympto- 
matology mention  was  made  of  cases  in  which  an  attack  can  be  cut  short 
by  quick  constriction  of  a  limb  when  the  aura  appears  in  that  limb.  I 
also  mentioned  a  patient  whose  seizure  was  arrested  at  the  very  onset  as 
soon  as  he  put  some  food  in  his  mouth.  An  attack  can  also  be  checked 
by  inhalation  of  a  few  drops  of  amyl  nitrite.  If  in  spite  of  these  means 
the  attack  continues,  it  is  advisable  not  to  interfere  with  it  except  in  so 
far  as  to  watch  that  no  injury  should  occur  to  the  patient  when  he  falls. 
In  view  of  the  disturbed  circulation,  it  is  urgent  to  loosen  the  clothes, 
collars,  neckties  and  the  end  of  a  folded  towel,  of  handkerchief  or  else  a 
piece  of  wood  be  placed  between  the  jaws  so  as  to  avoid  biting  of  the  tongue. 
When  sleep  supervenes,  the  patient  should  not  be  awakened,  as  otherwise 
the  post-epileptic  depression  increases. 

CHOREA  (OF  SYDENHAM) 

(St.  Vitus  Dance) 

Symptoms. — The  onset  may  be  rapid  or  gradual.  The  former  follows 
usually  a  severe  shock.  In  the  majority  of  cases  the  symptoms  develop 
gradually.  A  few  prodromal  symptoms  precede  the  appearance  of  the 
characteristic  symptoms.  The  patient  (who  is  usually  a  child)  becomes 
irritable,  morose,  inattentive.  Gradually  he  gets  restless  and  awkward 
movements  are  noticed  in  the  arms  and  legs.  Objects  fall  out  of  his 
hands,  grimaces  are  noticed  on  his  face. 

The  characteristic  choreic  symptoms  may  begin  in  one  leg  or  in  one 
arm.  They  soon  become  generalized.  The  patient's  musculature  is 
continuously  contracting.  The  movements  are  involuntary,  irregular  and 
incoordinate.  When  the  upper  extremity  is  affected,  he  is  unable  to  take 
hold  of  an  object  and  keep  it  for  a  certain  length  of  time,  is  unable  to  feed 
himself,  to  write.  In  attempt  to  approach  his  hand  to  an  object,  a  series 
of  various  incoherent  movements  will  be  produced  before  the  hand 
reaches  it.  The  fingers  separate,  approach,  flex,  extend.  The  entire 
limb  supinates,  pronates,  is  abducted  or  adducted.  The  shoulder  is 
raised,  lowered,  pushed  backward  or  forward.  The  leg  is  in  constant 
motion,  moves  in  every  direction  when  the  patient  is  at  rest.     The  toes 


496     •  CHOREA 

flex,  extend,  the  foot  turns  inward,  outward,  the  legs  bend  or  extend. 
When  seated  the  patient  crosses  his  knees,  approaches  or  separates  them. 
The  gait  is  also  irregular.  The  face  is  continuously  agitated,  so  that 
various  expressions  are  assumed  by  the  patient.  He  closes  and  opens 
his  eyes,  rolls  the  eyes  in  every  direction,  the  head  rotates,  the  lips  pout. 
Continuous  contraction  of  individual  muscles  of  the  face  and  forehead  is 
noticed  one  after  another.  The  tongue  is  continuously  moving  from  side 
to  side,  forward  and  backward.  The  speech  is  disturbed  because  of 
irregular  contractions  of  the  respiratory  muscles,  especially  of  the  dia- 
phragm. The  articulation  of  words,  emission  of  sounds,  is  difficult. 
Ziemsen  observed  with  the  laryngoscope  irregular  movements  of  the 
vocal  cords.  When  the  muscles  of  the  palate  and  pharynx  are  affected, 
deglutition  is  difficult.  The  muscles  of  the  trunk  and  pelvis  participate 
in  the  movements. 

To  sum  up,  the  muscles  of  the  entire  body  are  in  a  state  of  frequently 
interrupted  involuntary  contractions,  which  are  rapid,  irregular,  pur- 
poseless. 

Voluntary  movements  increase  the  contractions,  although  a  reverse 
condition  may  also  be  observed.  Emotion,  excitement,  may  increase 
the  twitchings,  but  sometimes  they  have  an  inhibitory  effect.  In  writing 
the  following  conditions  may  occur:  (1)  The  power  of  control  over  the 
movements  may  be  complete.  (2)  General  choreic  movements  may  be 
very  slight  or  else  much  incoordination  may  be  present. 

The  movements  usually  disappear  during  sleep. 

Sensory  symptoms  may  be  present  in  the  form  of  parassthesiae.and 
even  tenderness  of  the  muscles.  Diminished  objective  sensibility  is 
quite  common. 

Chorea  is  frequently  accompanied  by  the  following  symptoms. 

The  reflexes  may  be  normal,  increased  or  diminished. 

W.  Gordon  described  the  following  phenomenon.  When,  the  patient 
being  in  dorsal  position,  a  short  blow  is  struck  over  the  patellar  tendon,  the 
leg  will  at  first  respond  like  in  an  ordinary  knee-jerk,  but  instead  of  coming 
down  immediately  after,  will  remain  suspended  in  the  air  some  time  and 
gradually  come  down.  It  is  probably  due  to  a  prolonged  contraction  of 
the  quadriceps  muscle.  This  reflex  is  not  absolutely  constant,  but  it 
has  never  been  observed  outside  of  genuine  chorea. 

The  pupils  are  often  dilated.  Inequality  of  pupils  and  hippus  has 
been  observed.  Occasionally  optic  neuritis  is  met  with,  a  fact  which 
led  to  the  view  that  acute  chorea  is  of  infective  origin. 

The  pulse  is  rapid  and  quite  frequently  a  mitral  lesion  is  present. 

Urea  is  increased.     Phosphaturia  is  present. 


CHOREA  497 

The  mental  faculties  are  sometimes  involved.  Mental  dulness, 
diminished  attention,  weakness  of  memory,  excitability  or  else  depression 
are  not  infrequent.  In  exceptional  cases  a  delirium  with  confusion  and 
hallucinations  may  develop.  In  some  cases  the  mental  symptoms  may 
become  permanent  and  develop  into  dementia. 

The  general  nutrition  suffers  when  the  muscles  of  deglutition  are 
involved. 

Forms  of  Chorea. — Sydenham's  chorea  may  be  pronounced  and  very 
slight.  Between  these  extreme  forms  there  are  many  intermediary  forms. 
It  may  affect  one  side  of  the  body  and  in  then  called  hemichorea.  When 
there  is  a  marked  weakness  or  a  paretic  condition  of  the  extremities,  it  is 
called  paralytic  chorea. 

The  loss  of  power  and  of  voluntary  movements  in  paralytic  chorea 
may  be  generalized  or  confined  to  one  or  two  extremities  (mono-,  hemi-, 
or  paraplegic  forms).  The  paralysis  is  flaccid  and  there  is  no  involvement 
of  reflexes,  of  sensations  or  of  sphincters.     The  prognosis  is  good. 

In  the  pronounced  or  grave  variety  of  chorea  the  twitchings  are  so 
intense  that  the  patient  is  obliged  to  be  in  bed.  I  have  seen  patients 
whose  movements  were  so  violent  that  they  were  thrown  out  of  bed.  In 
such  cases  walking  is  impossible.  The  agitation  continues  even  during 
sleep.  The  violent  muscular  contractions  lead  to  traumata  and  such  a 
patient  is  often  covered  with  ulcerations.  Mental  disturbances  frequently 
accompany  grave  chorea.     Death  is  the  usual  termination. 

Chorea  of  pregnancy  occurs  usually  in  primiparas  during  the  first  half 
of  pregnancy.  It  is  a  grave  affection,  as  the  muscular  twitchings  are 
very  severe  and  interfere  with  sleep.  It  is  frequently  complicated  by 
cardiac  diseases  with  fever,  also  mental  disturbances.  The  gravity  of 
this  form  of  chorea  lies  also  in  the  spontaneous  interruption  of  pregnancy. 

Course,  Termination,  Prognosis.— The  average  case  of  Sydenham's 
chorea  lasts  about  one  or  two  months.  Periods  of  amelioration  and 
aggravation  are  observed  in  the  course  of  the  disease.  Recovery  is  the 
usual  result.  The  younger  the  child  the  better  is  the  prognosis.  Chorea 
occurring  in  youths  presents  a  serious  outlook,  as  it  lasts  longer  and  has  a 
tendency  to  become  chronic.  The  grave  form  of  chorea  has  an  un- 
favorable prognosis:  death  is  due  either  to  exhaustion  or  to  the  mental 
symptoms.  The  more  marked  the  latter  are,  the  more  serious  the  out- 
look is. 

Chorea  of  pregnancy  is  a  serious  malady  (see  above). 

Recurrences  are  very  frequent  in  chorea.  After  the  patient  has  made 
apparently  a  complete  recovery,  a  shock,  an  emotion,  promptly  brings 
on  another  attack.  Some  patients  have  every  year  an  attack  of  chorea 
32 


498  CHOREA 

during  several  years.  Chorea  is  sometimes  associated  with  other  neuroses, 
viz.  hysteria,  epilepsy. 

Diagnosis. — The  rapidity,  irregularity  and  incoordination  of  muscular 
contractions  are  sufficiently  characteristic  signs  for  diagnosis.  Occasion- 
ally hesitation  is  experienced  and  the  only  affections  with  which  chorea 
may  sometimes  be  confounded  are:  tic,  myoclonia,  athetosis  and 
Hysteria. 

Tic  and  Myoclonia  are  recognized  from  the  suddenness  and  instan- 
taneity  of  twitchings  which  are  confined  only  to  a  certain  portion  of  the 
body.  In  tic  the  movements  are  coordinate  and  purposive,  while  in 
chorea  they  are  distinctly  incoordinate.  The  tic  movements  can  be 
controlled  to  a  certain  extent,  choreic  movements  cannot  be  controlled. 
The  former  are  not  disabling,  the  latter  are. 

In  Athetosis  the  movements  are  slow  and  regular  and  affect  mostly 
the  fingers  or  toes.  In  Hysteria  the  movements  are  coordinate  and  they 
usually  develop  suddenly  after  an  emotion  or  during  an  hysterical 
paroxysm. 

Etiology. — Sydenham's  chorea  is  a  disease  of  young  age  and  affects 
children  from  the  age  of  seven  to  puberty.  Girls  are  more  predisposed 
than  boys.  It  may,  however,  occur  at  any  age.  The  predisposing 
causes  are  a  neuropathic  hereditary  tendency  which  can  be  traced  in  the 
majority  of  cases,  constitutional  diseases  (tuberculosis,  etc.),  anaemia  or 
a  debilitated  state  originating  from  any  cause. 

Infectious  diseases,  among  which  acute  inflammatory  rheumatism 
occupies  the  first  place,  are  not  infrequently  accompanied  or  rather 
followed  by  chorea.  Scarlet  fever,  measles,  diphtheria,  smallpox  may 
be  followed  by  chorea.  In  secondary  syphilis  chorea  has  been  observed 
and  the  former  may  be  the  cause  of  the  latter.  Recently  Milian  (Bull, 
et  Mem.  de  Soc.  med.  d.  hop.  Paris,  5  Dec,  191 2)  reported  fifteen  cases 
in  which  stigmata  of  hereditary  syphilis  were  present.  Moreover,  in 
eight  cases  out  of  thirteen  Wassermann  reaction  was  positive.  He  calls 
attention  to  von  B  okay's  case  in  which  Salvarsan  cured  the  chorea. 
Rheumatism,  however,  is  most  frequently  followed  by  chorea.  The  car- 
diac condition  which  is  so  frequently  met  with  in  chorea  is  probably  due 
to  the  preexisting  rheumatic  affection. 

Pregnancy  is  an  important  factor  in  causation  of  chorea,  especially 
in  cases  with  a  history  of  previous  attacks  (see  above). 

Fright,  emotion,  traumatism  are  frequently  the  immediate  causes  of 
chorea,  especially  in  predisposed  individuals.  Predisposed  children  being  in 
company  of  choreic  ones  may  sometimes  develop  the  disease  by  imitation. 

Chorea  is  rarely  observed  in  negroes. 


CHOREA  499 

Pathology. — Hyperemia,  punctiform  hemorrhages  and  perivascular 
alterations  have  been  observed  by  some  writers.  Hudovernig  (see 
below)  found  cellular  changes  analogous  with  those  of  anterior  polio- 
myelitis, especially  in  thalamus  opticus.  Proliferation  of  connective 
tissue  was  observed  in  the  spinal  meninges.  It  is  possible  that  these 
changes  are  due  to  a  toxi-infectious  process.  There  are  also  many 
observations  on  record  with  absolutely  negative  findings. 

Bignami  and  Nazari  (Presse  Med.,  191 1)  report  two  cases  of  hemi- 
chorea,  in  one  of  which  was  found  a  solitary  tubercle  in  the  left  superior 
cerebellar  peduncle,  in  the  other  a  hemorrhagic  focus  in  the  same  spot 
as  in  the  first  case. 

Pathogenesis  of  Chorea. — The  post-mortem  investigations  present 
nothing  definite  for  the  localization  of  the  disease.  There  is  a  tendency 
at  the  present  time  to  consider  chorea  as  infectious  in  nature.  The 
bacteriological  works,  especially  of  Pianese,  favor  this  view.  This 
author  found  in  the  spinal  cord  a  bacillus  with  the  cultures  of  which  he 
made  successful  inoculations.  Microorganisms  in  the  brain  were  also 
found  by  other  authors.  Poynton  and  Paine  isolated  from  the  cerebro- 
spinal fluid  a  diplococcus  which  after  an  inoculation  into  a  rabbit  produced 
muscular  twitchings.  The  diplococcus  was  found  in  the  pia-mater  and 
brain  in  choreic  patients  and  in  the  rabbits. 

The  cytological  examination  of  the  cerebro-spinal  fluid  showed  in  a 
number  of  instances  a  marked  lymphocytosis.  In  favor  of  the  infectious 
origin  speaks  also  the  occurrence  of  chorea  with  infectious  diseases.  I  have 
seen  cases  in  which  chorea  developed  subsequently  to  a  localized  inflam- 
matory focus;  the  twitchings  appeared  at  the  time  of  formation  of  pus. 
The  choreic  movements  disappeared  with  the  removal  of  the  infectious 
focus  (/.  Amer.  Med.  Ass'n.,  1910).  That  acute  chorea  is  due  to  an  in- 
fectious agent  is  highly  probable.  In  this  respect  it  is  analogous  to 
acute  anterior  poliomyelitis  with  this  difference,  that  in  the  latter  the 
motor  cells  are  being  destroyed,  hence  the  paralysis;  in  the  former  the 
motor  cells  of  the  cortex  and  of  the  nuclei  of  the  medulla  undergo  irri- 
tation, hence  the  twitching.  The  destructive  effect  in  the  latter  and  the 
irritating  effect  of  the  former  point  to  a  difference  in  the  virus  in  the  two 
affections.  Hudovernig's  pathologic  report  (Arch.  f.  Psych.,  1903)  shows 
a  very  great  resemblance  between  the  cellular  changes  of  both  affections. 
It  must  not  be  forgotten  that  the  bacteriological  investigations  cannot 
as  yet  be  accepted  as  absolutely  conclusive. 

Charcot's  and  Joffroy's  views  cannot  be  neglected.  According  to 
them  there  is  an  inherent  degenerative  predisposition  of  the  motor 
apparatus,  which  is  brought  in  evidence  as  soon  as  some  special  cause 


500  CHOREA 

disturbs  the  latter.  This  cause  may  be  an  acute  inflammatory  rheumatism 
or  any  infectious  disease. 

Treatment. — It  was  mentioned  above  that  a  neuropathic  tendency  is 
found  in  the  majority  of  cases.  The  indication  is  therefore  to  improve 
the  general  condition  of  the  patient  by  proper  dietetic  and  hygienic 
measures.  In  cases  of  anemia,  tuberculosis  or  other  constitutional 
diseases  an  effort  should  be  made  to  improve  these  conditions.  In  an 
average  case  of  chorea  factors  leading  to  emotion,  excitement  or  depression 
must  be  removed.  Mental  strain  is  contraindicated.  The  child  should 
not  be  sent  to  school.  The  patient's  life  should  be  so  regulated  as  to 
give  him  a  sufficient  amount  of  rest,  good  sleep,  proper  and  nutritious 
food  in  moderate  quantities.  I  am  in  a  habit  to  remove  meats  and  all 
stimulants,  including  tea  and  coffee.  Elimination  must  be  taken  proper 
care  of.  An  occasional  purgative  is  beneficial.  When  the  twitching  is 
marked  and  does  not  show  any  tendency  to  improve,  I  keep  the  patient 
in  bed.  This  procedure  has  given  me  excellent  results  in  many  obstinate 
cases.  Cool  spongings  followed  by  a  gentle  massage  have  a  sedative 
action  on  the  patient's  nervous  system.  In  cases  with  violent  twitch- 
ings  the  patient  must  be  guarded  against  unjury  and  being  thrown  out 
of  bed. 

Among  all  the  medications  the  most  reliable  ones  are:  arsenic  and 
antipyrin.  It  is  advisable  to  commence  the  treatment  with  the  first. 
Its  administration  should  begin  with  very  small  doses  and  only  very 
gradually  increased,  as  otherwise  intolerance  will  be  exhibited  very  early. 
An  average  child  of  ten  should  be  given  TTtiii  t.  i.  d.  of  Fowler's  solution 
during  the  first  two  or  three  days.  If  after  two  or  three  weeks  of  treatment 
no  improvement  is  noticed,  the  arsenic  should  be  substituted  by  antipyrin. 
To  a  child  of  the  same  age  gr.  j  of  the  latter  can  be  given  every  two  hours. 
I  have  frequently  obtained  very  good  results  with  antipyrin  when  arsenic 
failed.  When  none  of  these  drugs  yields  results,  the  patient  should  be 
kept  in  bed  and  given  bromides.  The  latter  may  be  given  conjointly 
with  arsenic. 

When  there  is  a  history  of  rheumatism,  sodium  salicylate,  aspirin  or 
salophen  give  very  satisfactory  results.  As  to  individual  doses  of  all 
medicaments,  they  will  have  to  be  modified  according  to  the  age  and  the 
intensity  of  symptoms,  but  it  is  always  wise  to  commence  with  very  small 
doses.  A  very  gradual  increase  in  the  amount  is  always  preferable  to  a 
rapid  increase,  as  it  establishes  a  satisfactory  tolerance. 

The  patient  must  be  frequently  observed,  and  with  the  first  signs  of 
physiological  intolerance,  the  given  drug  must  be  immediately  discon- 
tinued and  in  a  day  or  two  substituted  by  another. 


CHOREA  5OI 

Among  other  drugs  chloral  hydrate  is  to  be  recommended  for  con- 
trolling the  twitching  when  other  remedies  fail. 

In  chorea  of  pregnancy  artificial  termination  of  pregnancy  may  be 
considered.  The  latter  should  be  undertaken  when  the  indications  are 
strong,  viz.  when  life  is  endangered  by  exhaustion,  cardiac  or  renal 
lesions  or  mental  disturbances. 

The  latest  researches  of  Loeb  and  J.  B.  MacCallum,  also  of  W.  G. 
MacCallum  and  C.  Voegtlin,  show  a  certain  relationship  between  various 
twitchings  and  calcium  metabolism  also  the  function  of  the  parathyreoid 
glands.  A  trial  of  calcium  salts  and  of  parathyreoids  in  chorea  is  there- 
fore indicated. 

Finally  Marinesco  (Semaine  med.,  1908),  advises  the  use  of  intra- 
spinal injections  of  magnesium  sulphate  (25  per  cent.).  He  withdraws 
first  a  certain  amount  of  cerebro-spinal  fluid  and  injects  the  same  amount 
of  the  drug.  The  amount  injected  is  1  c.c.  to  each  25  pounds  of  bodily 
weight.  Marinesco  obtained  very  satisfactory  results  in  every  one  of 
his  cases.  He  advises  against  the  use  of  this  drug  in  grave  cases,  in 
chorea  of  pregnancy  and  in  cases  of  chorea  dependent  on  an  organic 
disease  of  the  central  nervous  system. 

CHRONIC  CHOREA 

HEREDITARY  CHOREA 

(Huntington's  Chorea) 

This  disease,  which  has  no  relation  whatever  to  Sydenham's  chorea, 
was  known  before  Huntington,  but  the  latter  was  the  first  to  call  special 
attention  to  three  important  elements  of  the  affection,  viz.  heredity, 
onset  at  the  age  of  thirty  or  forty  and  mental  symptoms. 

Symptoms. — The  clinical  picture  differs  little  from  that  of  Sydenham's 
chorea.  Like  in  the  latter,  the  movements  are  arhythmical,  irregular, 
incoordinate.  The  onset  is  slow  and  the  twitchings  at  first  appear  on  the 
lower  half  of  the  face.  Gradually  they  spread  to  the  upper  and  lower 
extremities,  also  the  trunk.  When  the  muscles  of  the  palate  and  pharynx 
become  involved,  the  deglutition  is  difficult.  The  tongue  is  particularly 
affected,  so  that  the  speech  becomes  indistinct  and  nasal  in  tone.  When 
the  diaphragm  is  involved,  the  respiration  is  disturbed.  The  reflexes 
are  exaggerated.  Muscular  weakness  is  usually  present,  but  there  is  no 
atrophy;  neither  are  there  changes  in  the  electrical  reactions.  Sensations 
are  intact. 

The  distinguishing  features  of  Huntington's  chorea  are: 


502  CHOREA 

i.  It  occurs  in  adult  life. 

2.  The  movements  are  slower  and  not  as  frequent  as  in  Sydenham's 
chorea. 

3.  The  muscles  of  the  eyeglobes  are  usually  not  involved. 

4.  The  upper  part  of  the  face  is  rarely  affected. 

5.  The  gait  is  characteristic.  It  is  analogous  to  that  of  an  inebriate. 
The  patient  makes  a  few  rapid  and  awkward  steps,  then  stops  suddenly; 
leans  forward,  looks  at  the  ground  and  then  again  advances  with  small 
steps.     All  this  is  done  rapidly  and  with  variation. 

6.  Voluntary  effort  may  repress  the  twitchings,  so  that  at  that  time 
the  patient  is  able  to  execute  delicate  acts,  as  writing  or  threading  a 
needle. 

7.  Rest  decreases  the  intensity  of  the  twitchings. 

8.  As  the  disease  advances  the  mentality  suffers.  Extraordinary 
irritability  is  constant  and  is  frequently  one  of  the  earliest  symptoms. 
Defective  power  of  attention  in  execution  of  physical  as  well  as  of  mental 
acts  is  one  of  the  most  essential  characteristics.  Gradually  the  memory 
for  recent  and  old  events  weakens  and  the  conceptions  become  retarded. 
The  patient  is  depressed  and  the  intellectual  faculties  become  feeble. 
There  is  a  tendency  to  suicide.  Dementia  is  the  ultimate  result.  The 
mental  phenomena  as  a  rule  follow  the  motor  phenomena,  but  in  some 
cases  they  precede. 

Course,  Termination,  Prognosis. — The  disease  lasts  many  years, 
develops  slowly,  but  it  is  essentially  progressive.  Death  occurs  either 
from  disturbance  of  deglutition  and  respiration  or  from  the  extreme  mental 
hebetude  or  else  from  some  intercurrent  disease. 

Diagnosis. — The  differential  diagnosis  with  Sydenham's  chorea  is 
given  above. 

In  tic  the  movements  are  abrupt  and  always  the  same. 

Etiology. — In  the  majority  of  cases  there  is  a  direct  heredity  or  a 
general  neuropathic  taint.  There  are  cases  on  record  in  which  several 
successive  generations  were  affected  with  this  disease.  Huntington 
observed  that  when  one  member  of  an  affected  family  escapes,  his  off- 
spring are  free  from  the  disease.  In  other  cases  there  may  be  a  family 
history  of  epilepsy  or  hysteria. 

Both  sexes  are  equally  affected.  The  disease  occurs  in  adult  life 
between  thirty  and  forty.  Emotions  have  a  very  important  influence 
upon  the  development  of  the  malady.  Traumata  and  pregnancy  are 
equally  exciting  causes. 

Pathogenesis  of  Huntington's  Chorea. — Post-mortem  examinations 
show  an  anatomical  basis  of  the  disease.     Atrophy  of  the  cortex  and  espe- 


ATHETOSIS  503 

daily  of  the  motor  area,  thickening  and  adhesions  of  the  meninges,  diffuse 
meningoencephalitis,  have  been  found.  Microscopically  disseminated 
foci  of  round  cells  in  the  cortex  and  white  matter  have  been  seen  quite 
frequently.  Whether  a  parenchymatous  degeneration  of  the  neurons  or 
a  vascular  alteration  is  the  primary  condition,  it  is  difficult  to  say.  There 
is  a  possibility  of  hereditary  malformation  of  the  central  nervous  system. 
The  relation  between  the  anatomical  findings  and  the  clinical  picture  of 
the  disease  is  not  entirely  established. 

Treatment. — When  treated  early  the  patients  may  derive  some  benefit 
from  good  hygienic  and  dietetic  measures,  from  bromides,  arsenic,  chloral, 
antipyrin.  As  a  rule  all  these  means  are  only  palliative.  The  disease  is 
progressive  and  incurable. 

ATHETOSIS 

It  is  characterized  by  continuous,  slow,  involuntary  movements, 
mostly  of  fingers  and  toes,  occurring  even  during  sleep. 

Unilateral  Athetosis  (Hemiathetosis)  stands  in  close  relation  to  hemi- 
plegia (see  this  chapter). 

Bilateral  Athetosis. 

This  is  mostly  a  congenital  condition  appearing  in  infancy  and  accom- 
panied by  mental  symptoms. 

Symptoms. — The  onset  may  be  insidious  or  rapid.  In  the  latter  case 
it  is  preceded  by  a  fright,  or  a  convulsive  seizure.  In  the  majority  of 
cases  the  course  is  progressive,  affecting  the  face,  extremities  and  trunk 
in  successive  order.  The  muscular  movements  are  involuntary,  slow, 
of  wide  range.  When  on  the  face,  expressions  of  fright,  joy,  laughing  or 
crying,  of  contemplation,  etc.,  will  be  alternately  observed.  The  eye- 
globes,  tongue,  usually  participate. 

In  the  upper  extremities  the  fingers  are  mostly  affected.  There  will 
be  a  continuous  display  of  flexion  and  extension,  also  of  abduction  and 
adduction.  Sometimes  the  wrist,  forearm  and  arm  are  similarly  affected. 
The  functional  disability  is  therefore  evident.  In  the  lower  extremity  the 
toes  and  ankle  are  mostly  involved.  When  the  neck  is  affected,  there  will 
be  an  oscillation  of  the  head  in  all  directions.     The  trunk  is  rarely  invaded. 

Rigidity  of  the  muscles  is  another  characteristic  feature  of  the  disease. 
It, becomes  marked  upon  a  voluntary  act.  The  spastic  condition  causes 
deformities  of  the  limbs  and  this  is  especially  marked  in  the  lower  extremi- 
ties. The  gait  is  spastic  and  difficult.  The  reflexes  are  exaggerated. 
When  the  muscles  of  the  lips  and  tongue  are  affected,  the  speech  is  diffi- 
cult ;  it  is  usually  slow  and  dragging.     The  muscles  are  hypertrophied  from 


5°4 


ATHETOSIS 


continuous  movements,  but  there  are  no  changes  in  their  electrical  re- 
actions. Relaxation  of  the  joints  and  subluxation  of  the  phalanges  are 
sometimes  observed. 


Fig.  151. — Position  of  Fingers  in  Athetosis.     (Slriimpell.) 


ATHETOSIS  505 

Mental  symptoms  are  the  third  characteristic  sign  of  double  athetosis. 
They  are  congenital  and  noticeable  early  in  life.  They  consist  of  a  feeble- 
ness of  all  the  intellectual  faculties.  In  exceptional  cases  the  intelligence 
is  preserved. 

Course,  Prognosis. — As  all  other  functions  of  the  body  are  intact,  the 
disease  does  not  endanger  life.  Death  occurs  from  some  intercurrent 
affection.     The  disease  itself  is  stationary  and  lasts  many  years. 

Pathogenesis. — The  lesions  found  post-mortem  have  not  been  con- 
stant, so  that  positive  conclusions  as  to  the  nature  of  the  disease  cannot 
be  drawn.  Cortical  lesions,  malformation  of  convolutions,  pachymen- 
ingitis, asymmetry  of  the  hemisphere,  of  the  cerebellum,  of  the  medulla, 
cerebral  sclerosis,  have  all  been  observed  in  cases  with  positive  findings. 
There  are  also  cases  in  which  the  central  nervous  system  was  found  to  be 
normal.  The  consensus  of  opinion  is  that  bilateral  athetosis  is  caused  by 
a  bilateral  irritation  of  the  motor  area  or  the  motor  pathway. 

Etiology. — A  hereditary  neuropathic  taint  is  present  in  the  majority 
of  cases.  Syphilis,  alcoholism,  epilepsy,  insanity,  etc.,  are  not  infre- 
quently traced  in  the  family. 

Premature  or  difficult  labor  is  a  potent  factor  in  causation  of  cerebral 
disturbances  and  therefore  of  double  athetosis.  Infectious  diseases  and 
traumata  occurring  in  early  infancy  are  sometimes  the  direct  causes  of 
the  disease.  In  some  cases  no  appreciable  cause  can  be  found.  The  dis- 
ease occurs  in  early  infancy,  although  later  development  has  also  been 
observed. 

Treatment. — Sedative  medications  (bromide,  chloral),  hydrotherapy, 
may  be  tried,  but  very  little  can  be  expected  from  them. 

Systematic  exercises,  with  the  purpose  of  controlling  the  movements, 
kept  up  for  a  long  time  and  carried  out  persistently  and  patiently,  may 
yield  satisfactory  results. 

DYSTONIA  MUSCULORUM  DEFORMANS  (DYSBASIA  LORDOTICA 

PROGRESSIVA) 

Oppenheim  in  191 1  (Neurolog.  Centralblatt.)  described  a  symptom- 
group  which  he  characterized  as  "a  disturbance  of  muscle  tone."  Prior 
to  him  Ziehen  (Allg.,  Ztschr.  f.  Psych.,  LXVIII)  observed  an  analogous 
condition  to  which  he  gave  the  name  of  "tonic  torsion  neurosis."  The 
disorder,  according  to  Oppenheim,  has  some  resemblance  to  Huntington's 
chorea  and  more  to  athetosis,  so  that  his  diagnosis  of  the  condition  at 
first  was  between  hysterical  lordosis  and  idiopathic  bilateral   athetosis. 

Symptoms. — The  main  features  of  the  disease  are :  a  deformity  about 


506 


ATHETOSIS 


the  pelvis  and  spasms  of  the  muscles  surrounding  the  pelvis,  also  twitchings 
in  other  muscles.  The  disease  may  begin  with  twitchings  in  the  upper 
limbs,  but  it  is  in  the  muscles  of  the  pelvic  girdle  that  they  are  most 
marked.  The  twitchings  are  evident  while  standing  or  walking,  but  not 
in  a  lying  position.  The  deformity,  which  is  persistent,  consists  of  a 
marked  lordosis  of  the  dorso-lumbar  region  with  a  lateral  inclination  of 
the  pelvis.     The  gait  is  especially  peculiar,  it  resembles  the  movements 


Fig.  152. — Dysb asia Lord otica  Progres- 
siva or  Dystonia  Musculorum  Deformans. 
(Oppenheim.) 


Fig.  153. — Same  as  Fig.  152. 


of  a  quadruped.  While  the  patient  walks,  he  is  affected  with  movements 
of  a  clownish  character ;  the  fatigue  and  strain  caused  by  such  movements 
brings  on  a  perspiration  and  rapidity  of  pulse.  The  muscular  twitchings 
are  either  a  rhythmical  tremor,  or  rhythmical  clonic  contractions,  especially 
in  the  lumbo-abdominal  muscles.  Tonic  contractions  are  seen  especially 
in  the  upper  extremities.  On  passive  movements  a  distinct  hypotonia  is 
observed  even  in  the  muscles  which  are  affected  with  tonic  contractions. 
(Figs.   152  and  153.) 


TIC  507 

The  disease  presents  no  evidences  of  organic  involvement  of  the  nerv- 
ous system.  The  tendon  reflexes  are  sometimes  diminished.  There  is  no 
paralysis,  no  muscular  atrophy,  no  electrical  change.  Sensations,  sphinc- 
ters, cranial  nerves,  psychic  sphere — are  all  intact.  The  course  is  progress- 
ive.    The  disease  occurs  in  children  from  eight  to  fourteen  years  of  age. 

Treatment. — Oppenheim  obtained  relief  from  metallotherapy  in  one 
case.  J.  Fraenkel  obtained  satisfactory  results  from  intraspinal  injections 
of  sulphate  of  magnesium  and  from  reeducation  movements. 

The  nature  of  the  disease  is  unknown. 

TIC 

It  consists  of  abrupt  involuntary  contractions  of  a  muscle  or  groups 
of  muscles. 

Symptoms. — The  sudden  involuntary  contraction  of  muscles  has  a 
convulsive  character  and  it  may  be  clonic  or  tonic.  In  the  first  case  the 
individual  contractions  are  separated  by  intervals  of  rest.  In  the  second 
case  the  contractions  are  so  near  each  other  that  they  give  the  impression 
of  a  prolonged  contracture.  Unlike  chorea  the  tic  is  characterized  by 
coordinate  movements,  by  systematized  movements.  At  the  beginning 
of  their  development  these  movements  consisted  of  muscular  con- 
tractions executed  for  a  certain  definite  purpose,  but  in  an  exaggerated 
manner.  For  example,  tic  of  the  eyelids  produces  exactly  the  act  of 
their  sudden  closure  done  to  protect  the  eye  from  penetration  of  a 
foreign  body.  Little  by  little,  when  these  movements  are  frequently 
repeated,  they  become  a  matter  of  habit  and  necessity.  Tic  is  therefore 
a  disease  of  habit,  a  habit  which  through  its  persistency  acquires  a 
morbid  character. 

Tic  may  affect  one  muscle,  if  this  muscle  by  itself  has  a  certain  func- 
tional purpose.  What  is  important  is  the  fact  that  the  invaded  area 
does  not  correspond  to  a  well-defined  anatomic  distribution  of  a  certain 
nerve  or  nerves  contrary  to  what  is  seen  in  spasm.  In  the  majority  of 
cases  several  muscles  contract  simultaneously,  as  their  associated  action 
is  necessary  for  execution  of  certain  acts.  Occasionally  a  certain  portion 
of  a  muscle  may  be  affected  by  tic;  it  occurs  in  those  muscles  various 
portions  of  which  have  different  functions  (deltoid,  trapezius,  etc.).  The 
form,  intensity  and  rapidity  of  the  twitching  vary  from  patient  to  patient 
and  in  the  same  patient.  Tic  has  a  tendency  to  spread  and  invade  other 
functions,  so  that  the  twitch  of  the  face  may  be  accompanied  by  a  sudden 
protrusion  of  the  tongue  or  by  a  laryngeal  noise,  by  a  scream  or  by  a 
certain  gesture  in  other  parts  of  the  body,  such  as  sudden  kick  of  the  foot. 


5o8 


TIC 


Gilles  de  la  Tourette  describes  various  tic  movements  accompanied  by 
coprolalia,  viz.  enunciation  of  profane  words.  Another  characteristic 
feature  of  tic  is  the  possiblitiy  with  a  certain  effort  of  retarding  or  sup- 
pressing the  muscular  contraction.  The  reason  of  it  lies  in  the  cortical 
nature  of  the  phenomenon. 

Tic  usually  disappears  during  sleep. 

Sensations ,  reflexes,  sphincters  are  intact  in  tic. 

The  affected  muscles  may  sometimes  become  hypertrophied,  but  this 
condition  is  only  functional  in  nature. 


Forms 

Tic  of  the  Face. — The  most  frequent  tic  of  all  the  muscles  of  the  face 
is  that  of  the  eyelids  (palpebral  tic).  It  is  usually  bilateral.  The  neigh- 
boring muscles  frequently  participate.  The  eye-globes  frequently  partici- 
pate (nystagmoid  tic).  The  latter  symptom  is  not  observed  in  blepharo- 
spasm. Next  to  the  eyelids  the  lips  are  the  most  frequently  involved. 
Grimaces  are  the  result  of  tic  of  the  lips. 


Fig.   154.  Fig.  155. 

Figs.  154  and  155  — Mental  Torticollis  and  Correction  Gesture.     (Brissaud.) 

Any  other  muscles  of  the  face,  including  the  platysma,  may  be  affected 
by  tic.  When  the  tongue  is  involved,  all  sorts  of  noises  may  be  heard 
from  the  movements  of  the  tongue.  When  the  masticatory  muscles 
suffer  from  tic,  abrupt  and  repeated  lowering  or  raising  of  the  lower  jaw 
will  be  noticed. 

Tic  of  the  Neck. — Sudden  and  repeated  rotation,  flexion  or  extension 
of  the  head  will  be  noticed  according  to  the  muscles  involved.  The 
flexors  are  more  frequently  affected  than  the  extensors.  Tic  of  the  neck 
is  frequently  associated  with  tic  of  the  face  or  of  the  shoulders. 


TIC 


509 


Torticollis  in  the  form  of  tic  (mental  torticollis  of  Brissaud)  or  spas- 
modic torticollis  may  present  itself  as  sudden  simple  rotatory  movements, 
rotation  with  flexion  or  rotation  with  extension.  The  tic  may  consist  of  a 
single  movement  or  of  a  series  of  successive  movements.  Among  all 
the  muscles  of  the  neck  the  sterno-mastoid  is  the  most  frequently  affected. 
During  the  contraction  the  face  is  turned  to  the  opposite  side,  the  head  is 
inclined  on  the  same  side  and  the  ear  touches  the  shoulder.  When  the 
upper  part  of  the  trapezius  and  splenius  muscles  are  involved,  together 
with  the  sterno-mastoid,  the  head,  besides  being  inclined,  is  also  drawn 
backward.     When  the  muscles  of  the  anterior  part  of  the  neck  are  in- 


Eig.  156. — Spasmodic  Torticolis.     Patient  Seen  in  Attempt  to  Correct  Position 

of  Head. 

volved  (which  is  rare),  the  head  falls  forward  so  that  the  chin  touches  the 
chest.  Rotatory  tic  of  the  head  is  often  combined  with  elevation  of  the 
shoulder. 

The  patients  frequently  complain  of  pain  or  of  a  drawing  sensation 
in  the  neck.  This  is  of  course  due  to  the  repeated  muscular  contractions. 
Rest,  physical  and  mental,  decreases  the  intensity  of  the  tic.  Fatigue 
and  emotions  increase  it.     The  patients  use  all  sorts  of  subterfuges  to 


510  TIC 

remove  the  torticollis.  The  most  frequent  attitude  is  to  keep  a  finger  on 
the  chin.  The  onset  of  the  affection  is  usually  insidious.  The  disease 
originates  from  some  false  position  adapted  at  first  for  the  purpose  of 
relieving  pain  in  the  neck  or  from  unavoidable  positions  in  certain  occu- 
pations which  require  special  gestures  or,  as  some  pretend,  from  visual 
disturbances.  The  act  is  repeated  a  number  of  times,  at  first  voluntarily, 
and  finally  involuntarily.  The  condition  is  thus  established.  In  cases  of 
long  standing  the  involved  sterno-mastoid  muscle  may  become  hyper- 
trophied,  and  the  muscle  of  the  opposite  side  atrophied  for  want  of 
exercise. 

Tic  of  the  shoulder  consists  mostly  of  a  sudden  raising  of  the  shoulder. 
The  habit  is  acquired  through  some  discomfort  felt  from  the  clothes 
covering  the  shoulder  or  else  from  some  pain  in  that  region. 

Tic  of  the  arms,  hands  and  fingers  occasionally  occurs. 

Tic  of  the  Trunk  is  a  rare  condition.  In  a  case  reported  by  me  (Amer. 
Medicine,  1906)  the  tic  consisted  of  abrupt  salutations:  the  recti  muscles 
of  the  abdomen  contracted. 

Tic  of  the  Lower  Extremities  may  be  manifested  in  sudden  bending 
of  the  knees,  jumping,  kicking,  changing  of  the  steps. 

Respiratory  Tic. — It  presents  itself  as  abrupt  inspiratory  or  expiratory 
movements.  It  is  frequently  associated  with  contractions  of  the  muscles 
of  the  naso-pharynx.     The  sounds  of  snuffing,  of  snoring,  are  heard. 

Laryngeal  Tic  is  manifested  either  in  sudden  laryngeal  sounds,  as 
grunting,  barking,  etc.,  or  shouting  certain  syllables  or  words  (Verbal 
Tic) .  The  latter  is  particularly  encountered  in  a  symptom-group  known 
under  the  name  of 

Tic  Convulsif. — This  condition  was  described  first  by  Guinon  and 
Gilles  de  la  Tourette.  It  begins  with  the  muscles  of  the  face.  Blinking 
of  the  eyes,  pouting  the  lips,  protruding  the  tongue,  grimacing  the  face, 
blowing,  whistling,  chattering  of  the  teeth — all  these  acts  are  done  with 
extreme  rapidity,  convulsive-like.  Frequently  the  tic  does  not  remain 
confined  to  the  face;  the  neck,  upper  and  lower  extremities  and  trunk 
participate.  Thus  the  patients  will  also  exhibit  sudden  raising  and  lower- 
ing of  the  shoulders,  propulsion,  retropulsion  or  lateral  movements  of  the 
trunk,  rubbing  of  the  hands,  raising  of  a  finger  to  the  nose,  ear  or  mouth, 
stamping  of  the  feet,  jumping,  dancing,  etc.  All  these  movements  are 
spasmodic,  abrupt,  rapid,  but  they  systematically  succeed  each  other. 
They  may  be  arrested  temporarily,  but  at  the  expense  of  a  painful  struggle 
and  intense  anxiety.  Voluntary  acts  arrest  the  movements.  They 
disappear  during  sleep. 

Another  characteristic  manifestation  of  tic  convulsif  is  coprolalia  or 


TIC  511 

echolalia.  The  first  consists  of  a  sudden  use  of  obscene  words  in  the 
midst  of  an  ordinary  conversation.  In  echolalia  the  patient  repeats  sounds, 
words  or  syllables  heard  around  him.  Sometimes  he  repeats  movements 
seen  by  him  (echokinesis) .  All  these  acts  are  done  under  the  influence 
of  an  irresistible  impulse.  The  patient  is  always  fully  conscious  of  his 
uncontrollable  movements.  To  mask  them  he  frequently  executes  some 
voluntary  acts  in  rapid  succession,  but  it  is  not  an  easy  task  to  over- 
come the  involuntary  movements.  For  the  same  reason  the  patient 
is  seen  doing  the  most  peculiar  acts,  as  spending  considerable  time  in 
counting  the  buttons  on  his  coat,  the  number  of  stores  on  the  street, 
his  own  footsteps,  etc.  Here  the  motor  phenomenon  of  Gilles  de  la 
Tourette's  disease  may  be  absent,  but  imperative  conceptions  compel 
the  patient  to  utter  certain  words  or  sentences  or  perform  certain  acts. 
This  is  a  Psychic  Tic. 

Tic  of  Salaam  (Spasm  nutans). — It  is  a  rare  affection  and  occurs 
mostly  in  infants.  It  consists  of  the  act  of  flexion  of  the  head  and  the 
upper  part  of  the  trunk  repeated  a  great  many  times — from  twenty  to 
fifty  in  a  minute.  It  is,  otherwise  speaking,  a  convulsive  salutation. 
The  affection  is  also  spoken  of  as  "head  nodding."  In  some  cases  the 
phenomenon  assumes  the  character  of  epilepsy.  Thus  it  may  be  preceded 
by  a  sudden  pallor  of  the  face,  staring  and  dilatation  of  the  pupils;  there 
may  be  also  a  loss  of  consciousness.  In  some  of  these  cases  genuine 
epileptic  convulsions  develop  later. 

Course,  Duration,  Prognosis. — The  tendency  of  tic  is  to  spread  and 
invade  other  portions  of  the  body.  The  duration  is  uncertain.  Recur- 
rences are  frequent.  The  younger  the  individual,  the  more  is  the  case 
hopeful  as  to  recovery.  Rest,  mild  diversion,  quit  life  have  a  beneficial 
effect.  Fatigue,  emotions,  aggravate  and  prolong  the  condition.  The 
longer  the  tic  lasts  the  more  obstinate  is  the  case.  Among  all  the  forms 
Tic  convulsif  has  the  gravest  prognosis.  It  has  an  essentially  progressive 
course.  The  mental  symptoms  which  accompany  the  motor  phenomena 
may  terminate  in  dementia. 

Tic  in  the  aged  presents  also  an  unfavorable  prognosis. 

The  other  forms  of  tic  are  amenable  to  treatment  if  properly  managed. 

Diagnosis. — Although  the  symptoms  of  tic  are  typical,  nevertheless 
it  may  be  confounded  with  some  other  affections.  It  is  spasm  that  is 
sometimes  difficult  to  differentiate  from  tic. 

In  tic  the  movements  are  purposive,  only  in  an  exaggerated  form.  In 
spasm  one  fails  to  find  the  least  tendency  to  reproduce  physiologic  acts.. 
Spasm  of  the  face,  for  example,  has  no  resemblance  to  ordinary  mimicry 
In  tic  the  muscles  involved  do  not  correspond  to  a  weD  -defined  anatomical 


512  TIC 

distribution  of  a  nerve.  In  spasm,  on  the  contrary,  the  convulsive 
movements  are  produced  solely  in  the  area  of  distribution  of  a  nerve. 
Tic  disappears  during  sleep,  spasm  does  not.  An  effort  of  will  or  attention 
is  capable  to  arrest  or  inhibit  tic,  but  not  a  spasm  of  the  muscles.  In 
the  majority  of  cases  of  tic  we  find  a  hereditary  predisposition  to  some 
nervous  disorder.     This  is  not  observed  in  cases  of  spasm. 

Paramyoclonus  multiplex  is  recognized  by  its  clonic  contractions, 
which  are  sudden,  quick  in  succession,  irregular,  arhythmical;  they  may 
involve  not  only  an  entire  muscle,  but  also  a  portion  of  it  and  usually 
they  affect  symmetrical  muscles.  They  occur  more  frequently  in  the 
lower  extremities  than  in  any  other  portion  of  the  body;  the  face  never 
participates.  They  cannot  be  controlled  by  the  patient.  They  can  be 
easily  brought  on  by  stimulation  of  the  skin. 

Myokymia  is  characterized  by  fine  fibrillary  contractions,  also  by 
vaso-motor  and  sensory  disturbances. 

In  chorea  the  movements  are  incoordinate,  irregular  and  not 
convulsive. 

Pathogenesis. — As  said  above,  tic  is  a  functional  disturbance  in  which 
repetition  of  the  act  is  influenced  by  an  imperative  desire,  so  much  so 
that  if  the  patient  resists  the  act  he  suffers,  but  after  the  movement  is 
produced  there  is  great  relief  and  satisfaction.  It  is  therefore  evident 
that  the  mentality  plays  a  certain  role  in  the  phenomenon.  It  is  because 
of  a  deficient  will  power  that  the  twitchings  become  automatic,  that  a 
habit  is  established.  In  fact  tic  occurs  usually  in  individuals  with  peculiar 
ideas,  desires  and  tendencies.  They  are  eccentric,  not  stable,  and  fre- 
quently develop  fixed  ideas,  obsessions.  For  this  reason  Brissaud  con- 
siders tic  as  a  "psychic"  malady. 

Etiology. — Tic  can  be  considered  as  a  degenerative  neurosis.  A 
neuropathic  heredity  plays  an  important  role.  Insanity,  epilepsy, 
hysteria,  tic,  tuberculosis,  diabetes,  organic  nervous  diseases,  are  not 
infrequently  traced  in  the  family  histories  of  individuals  affected  with  tic. 

Bad  hygienic  surroundings,  habits,  excesses,  undue  cerebral  fatigue, 
are  predisposing  causes  of  tic. 

Local  irritation  and  local  lesions  (eyes  and  naso-pharynx)  are  the 
exciting  causes. 

Tic  is  rare  in  very  young  children.  It  usually  occurs  at  the  age  when 
great  physiological  changes  take  place,  viz.  puberty,  menopause,  old  age. 

Treatment. — In  view  of  the  neuropathic  make-up  of  individuals 
suffering  from  tic,  the  first  indication  is  to  improve  their  general  health 
with  proper  hygienic  and  dietetic  measures.  Such  patients  must  lead  a 
life  free  from  excitement  or  emotions  of  any  sort. 


TIC  513 

As  to  the  tic  itself,  Brissaud,  Meige,  Feindel  and  others  obtained 
satisfactory  results  from  special  physical  and  psychic  methods.  The 
first  consists  either  of  voluntary  immobilization  followed  by  systematic 
exercises.  When  immobilization  is  used,  the  patient  is  taught  to  im- 
mobilize the  affected  muscles  for  a  gradually  increasing  period  of  time. 
The  sittings  are  held  daily,  at  first  only  for  a  few  seconds  and  only  two 
or  three  times  a  day.  Gradually  the  number  of  exercises  is  increased  and 
the  duration  of  each  is  prolonged.  Patience  and  perseverance  are  re- 
quired. A  child  must  be  placed  in  charge  of  some  trained  person.  An 
adult  can  be  taught  how  to  proceed.  He  is  advised  to  have  before  him 
a  mirror  so  that  he  can  watch  the  procedures.  When  a  certain  amount  of 
control  has  been  obtained  by  the  patient,  the  next  method  is  taken  up, 
viz.,  voluntary  movements. 

Exercises  consist  of  replacing  the  involuntary  movements  of  the  tic 
by  voluntary  and  correct  movements.  By  being  voluntarily  contracted, 
but  in  a  slow,  deliberate  and  correct  manner,  the  affected  muscles  fall 
under  control  and  thus  become  trained.  Here  again  the  first  seances 
and  their  duration  must  be  brief.  Gradually  they  are  increased.  These 
exercises  should  be  made  under  control  of  an  observer  or  the  patient  can 
train  himself  gradually  in  the  performance.  In  the  latter  case  he  places 
himself  before  a  mirror  and  is  thus  able  to  acquire  a  certain  skill  in  the 
treatment. 

The  psychic  method  plays  an  enormous  role.  It  consists  of  continuous 
encouragement  and  of  pointing  out  to  the  patient  the  necessity  of  training 
his  will  power  in  overcoming  the  involuntary  movements. 

Isolation,  rest  in  bed,  removal  from  usual  surroundings,  together  with 
the  above  physical  and  mental  training  will  give  in  a  number  of  cases 
satisfactory  results. 

As  to  medications  there  is  none  to  be  relied  upon.  The  usual  sedatives 
may  be  employed. 

The  most  recent  researches  of  Loeb  and  J.  B.  MacCallum,  also  of 
W.  G.  MacCallum  and  C.  Voegtlin,  show  a  certain  relationship  between 
abnormal  motor  phenomena  and  calcium  metabolism,  also  the  function 
of  the  parathyreoid  glands.  A  trial  of  calcium  salts  and  of  parathyreoids 
in  tic  is  therefore  indicated. 

In  spasmodic  torticollis  operative  procedures  have  been  attempted. 
They  consist  of  excision  of  a  portion  of  the  spinal  accessory  nerve  on  the 
affected  side  and  of  section  of  the  posterior  primary  divisions  of  the 
upper  cervical  nerves  on  the  opposite  side.  I  have  seen  failures  from  this 
operation. 

33 


514 


TIC 


FACIAL  SPASM 

Symptoms. — Spasm  of  the  face  commences  with  clonic  contractions 
which  as  they  advance  gain  in  rapidity  and  at  the  height  of  the  attack 
are  replaced  by  tonic  contractions.  As  the  latter  subside,  clonic  con- 
tractions reappear  and  remain  until  the  attack  is  over.  The  entire 
cycle  lasts  but  a  minute.  The  following  individual  features  are  observed 
during  the  paroxysm.  The  forehead  on  the  affected  side  is  wrinkled, 
the  orbicularis  palpebrarum  closes  the  eye;  there  is  a  simultaneous  con- 


Fig.  157. — Facial  Spasm  on  the  Left. 


traction  of  the  frontalis  and  orbicularis  palpebrarum.  This  phenomenon 
is  called  by  Babinski  paradoxical  synergia.  The  zygomatic  muscles 
deviate  the  angle  of  mouth.  The  nose  is  curved  toward  the  affected 
side  and  the  chin  presents  a  characteristic  depression  on  the  affected  side. 
The  muscular  contractions  may  be  either  fascicular,  tremulous  or  coarse, 
of  a  wider  range  (Figs.  157  and  158). 

The  muscles  involved  in  facial  spasm  correspond  to  the  well-defined 
anatomical  distribution  of  the  seventh  nerve  and  according  to  the  case 
the  convulsive  movements  predominate  in  the  upper  or  the  lower  portion 
of  the  nerve.  Facial  spasm  occurs  during  sleep.  No  effort  of  will  or 
attention  is  capable  to  arrest  or  prevent  an  attack. 


TIC 


515 


The  most  frequent  form  is  hemispasm,  but  double  spasm  has  also 
been  observed.  Meige  described  a  median  facial  spasm,  which  consists 
of  bilateral  convulsive  movements  predominating  near  the  median 
line  of  the  face.  Both  orbiculares  palpebrarum  are  most  markedly 
involved. 

Pathogenesis. — The  stimulation  of  the  muscles  supplied  by  the  seventh 
nerve  may  originate  in  the  seventh  nerve  itself  or  in  its  nucleus  or  else 
in  any  of  the  sensory  fibers  of  the  fifth  nerve.     Blepharospasm  is  an  ex- 


^f              ' '"  ''4&L     ^I^^^H 

""1 

B 

Fig.  158. — Facial  Palsy  on  the  Left  Produced  by  Injection  of  Alcohol  into  the 

Left  Facial  Nerve. 


ample  of  stimulation  of  the  orbicularis  through  an  irritation  of  the  sensory 
fibers  of  palpebral  mucosa.  Facial  spasm  has  been  observed  in  attacks 
of  neuralgia,  in  connection  with  carious  teeth.  Not  infrequently  the  spasm 
is  found  limited  to  both  upper  and  lower  eyelids.  It  is  called  blepharo- 
spasm. The  contractions  may  be  tonic  or  clonic.  In  the  first  variety 
the  eyelids  are  persistently  closed  during  the  paroxysm,  which  may  last 
many  minutes.  It  is  due  to  some  irritation  of  ocular  branches  of  the  fifth 
nerve.     The  clonic  variety  consists  of  rapid  winking  movements. 

The  majority  of  cases  show  that  the  cause  of  facial  spasm  lies  in  the 
peripheral  portion  of  the  seventh  nerve.  The  occurrence  of  facial  spasm 
in  cases  of  facial  palsy  speaks  in  favor  of  peripheral  cause  of  the  spasm. 


516  MYOCLONIA 

Neuroma  on  the  facial  nerve  has  been  found  in  such  cases  (A.  Thomas, 
Rev.  X enrol.,  1907).  While  an  irritation  of  the  peripheral  end  of  the  nerve 
is  the  most  common  cause,  nevertheless  facial  spasm  has  been  observed 
also  in  organic  lesions  of  the  central  nervous  system.  In  cases  of  meningo- 
encephalitis, of  pseudo-bulbar  palsy,  of  the  pons,  facial  spasm  has  been 
observed.  Finally  the  above-mentioned  cases  of  involvement  of  the 
fifth  nerve  show  that  a  lesion  of  any  portion  of  the  reflex  arc  (sensory 
fibers,  nucleus  of  the  seventh  nerve,  motor  fibers  of  the  facial  nerve)  may 
be  the  cause  of  facial  spasm. 

Course,  Duration,  Prognosis.- — It  is  usually  rebellious  to  medical 
treatment.  It  may  have  intermissions.  It  lasts  ordinarily  an  indefinite 
time.     It  has  no  effect  on  patient's  life. 

Treatment. — If  a  local  cause  of  irritation  can  be  detected,  its  removal 
is  necessary.  Freezing  of  the  face  on  the  affected  side  has  been  recom- 
mended by  Weir  Mitchell.  The  most  effective  method  of  treatment  is  in- 
jection of  a  few  minims  of  80  per  cent,  alcohol  into  the  nerve  at  its  exit  of 
the  stylomastoid  foramen.  It  has  given  me  the  most  gratifying  results. 
The  spasms  ceased  for  periods  ranging  from  eighteen  months  to  three 
years.  The  facial  palsy  which  follows  immediately  the  injection  disap- 
peared at  the  end  of  five  or  six  weeks  in  every  case.  In  some  cases 
returns  of  spasm  were  treated  with  repeated  injections.  No  bad  results 
followed  repetition  of  injections.  An  experimental  study  {Trans.  Amer. 
Neurol.  Ass'n..  1913)  on  dogs  has  shown  me  that  injection  of  alcohol 
into  a  motor  nerve  is  followed  by  very  slight  pathological  changes  in 
the  perineurium,  but  the  nerve  fibers  themselves  remain  unaffected. 

In  cases  of  double  facial  spasm  the  injection  should  be  made  into  each 
nerve  separately  and  only  after  the  palsy  disappeared  on  one  side.  Alco- 
holic injections  into  the  nerve  may  be  tried  even  in  cases  of  organic  diseases 
of  the  nervous  system. 

MYOCLONIA 

The  disease  is  characterized  by  sudden  unsystematized,  involuntary 
clonic  contractions  similar  to  those  produced  by  an  electric  shock.  They 
may  be  localized  or  disseminated.  The  following  varieties  belong  to  the 
group  myoclonia. 

I.  Paramyoclonus  [Multiplex  of  Friedreich. 
II.  Familial  Myoclonia  with  Epilepsy  of  Unverricht. 
III.  Myokymia. 

IV.  Electric  Chorea  of  Bergeron-Henoch. 
V.  Dubini's  Chorea. 


MYOCLONIA  517 

Pathogenesis  of  Myoclonias. — The  symptom  myoclonia  may  be  en- 
countered in  the  course  of  various  organic  and  functional  nervous  diseases. 
That  it  may  be  an  independent  affection  is  true.  That  it  may  be  merely 
a  hysterical  phenomenon  is  also  correct.  It  is  accepted  by  the  majority 
of  observers  that  myoclonia  is  to  be  considered  as  an  episodic  manifesta- 
tion in  neuropathic  individuals. 

As  to  the  physiological  basis  for  myoclonic  twitchings,  the  views  are 
divided.  The  majority  believe  that  the  condition  is  due  to  an  irritation 
of  the  cells  of  the  anterior  cornua  of  the  spinal  cord. 

The  latest  researches,  particularly  of  Loeb  and  J.  B.  MacCallum,  also 
of  W.  G.  MacCallum  and  C.  Voegtlin,  show  a  certain  relationship  between 
various  twitchings  and  calcium  metabolism,  also  the  function  of  the  para- 
thyreoid  glands. 

I.  Paramyoclonus  Multiplex 

Symptoms. — The  myoclonic  contractions,  which  are  sudden  and 
lightning-like,  appear  first  in  the  lower  extremities,  but  they  may  be- 
come generalized.  The  face  as  a  rule  is  respected.  The  contractions  are 
unequal,  irregular  and  arhythmical.  They  may  affect  individual  muscles 
or  groups  of  muscles.  Their  frequency  may  vary:  they  may  occur  every 
few  minutes  or  every  half  hour.  They  may  be  mild  or  so  violent  as  to 
move  the  affected  part.  When  in  the  lower  limbs,  the  locomotion  is 
disturbed.  When  in  the  upper  limbs,  movements  of  flexion,  extension, 
supination,  etc.,  are  observed  so  that  the  usual  occupation  of  the  patient 
is  impossible.  Usually  symmetrical  muscles  on  both  sides  of  the  body  are 
affected.  The  muscles  of  the  limbs  are  more  frequently  affected  than 
those  of  the  trunk. 

If  the  muscles  of  the  pharynx,  larynx  and  diaphragm  are  affected, 
disturbance  of  deglutition  and  of  respiration,  respectively,  will  be  observed. 

The  nutrition  of  the  muscles  as  well  as  their  electrical  reactions  are 
intact.     Sensations  are  normal.     The  reflexes  are  exaggerated. 

The  muscular  contractions  cease  during  sleep;  they  may  be  sometimes 
arrested  or  lessened  by  a  voluntary  effort,  although  in  some  cases  a  reverse 
condition  is  observed.     Emotion  increases  their  intensity  and  frequency. 

Course,  Duration,  Prognosis. — The  disease  is  progressive  and  its 
onset  is  insidious.  It  may  last  indefinitely.  Cases  of  recovery  have  been 
reported.     Recurrences  are  very  frequent. 

Diagnosis. — The  sudden  lightning-like  clonic  contraction  is  sometimes 
sufficient  for  the  diagnosis. 

In  chorea  the  movements  are  not  so  abrupt  and  their  range  is  wider 
than  in  myoclonia. 


518  MYOCLONIA 

It  is  with  tic  that  the  differential  diagnosis  presents  sometimes  great 
difficulties.  However  in  tic  the  movements  are  coordinate  and  systema- 
tized. 

Etiology. — Paramyoclonus  is  found  sometimes  associated  with  organic 
and  functional  nervous  diseases,  also  insanities.  A  neuropathic  heredity 
exists  in  the  majority  of  cases.  Infectious  diseases,  intoxications,  fatigue, 
emotion  and  traumatism  are  among  the  exciting  causes. 

Treatment. — When  the  condition  is  only  a  symptom  of  other  diseases, 
the  first  indication  is  to  treat  the  latter.  As  in  the  majority  of  cases 
myoclonia  develops  in  neuropathic  subjects,  much  attention  should  b'e 
given  to  the  general  health.  Hydrotherapy,  moderate  exercises,  proper 
mode  of  living,  avoidance  of  excitement  and  worry,  nutritious  food,  etc., 
are  necessary  (see  chapter  on  Neuropathy) .  For  controlling  the  muscular 
contractions  sedatives  may  be  employed,  viz.  bromides,  antipyrin, 
chloral  and  others.  Arsenic  has  been  advised.  Confinement  to  bed  may 
sometimes  be  of  service.     Not  much  can  be  expected  from  medications. 

The  above-mentioned  remarks  concerning  calcium  metabolism  and 
the  function  of  the  parathyreoid  glands  (see  Pathogenesis)  indicate  the 
use  of  calcium  salts  and  of  parathyreoids  in  myoclonias. 

II.  Familial  Myoclonia  with  Epilepsy 

(Unverricht's  Type) 

This  form  presents  an  association  of  paramyoclonus  multiplex  with 
epilepsy.  The  latter  may  occur  only  early  in  life  and  then  disappear,  to  be 
substituted  by  myoclonic  twitchings,  or  else  accompany  the  myoclonia. 
The  occurrence  of  the  same  condition  in  several  members  of  the  same 
family  is  a  striking  feature  of  the  disease.  In  Unverricht's  first  case 
(1.891)  five  brothers  and  a  sister  were  thus  affected. 

ILL  Myokymia 

It  is  characterized  by  continuous  fibrillary  contractions.  The  muscles 
of  the  extremities  are  most  frequently  involved,  although  other  parts 
of  the  body  may  be  also  affected.  Sometimes  pain  and  hyperhidrosis 
accompany  the  muscular  twitchings.  In  one  of  my  cases  myokymia  of 
the  right  lower  half  of  the  face  was  associated  with  myoclonia  of  the  upper 
half  of  the  face.  The  least  mechanical  irritation  increased  the  contrac- 
tions. The  affected  muscles  presented  a  decreased  faradic  and  galvanic 
irritability. 


TETANY  519 

IV.  Electric  Chorea  (Bergeron-Henoch) 

It  consists  of  sudden  twitchings  rapidly,  but  rhythmically,  repeating 
themselves.  An  attempt  to  control  them  increases  their  intensity. 
They  disappear  during  sleep. 

They  may  affect  any  portion  of  the  body.  The  movements  are  so 
frequent  and  intense  that  the  patient  is  obliged  to  give  up  his  usual  work. 
The  nutrition  of  the  affected  muscles  is  not  disturbed.  The  prognosis 
is  usually  good. 

In  a  large  number  of  cases  the  disease  was  associated  with  gastric 
disturbances  and  improvement  of  the  latter  was  followed  by  disappear- 
ance of  the  muscular  twitching.  Autointoxication  is  therefore  supposed 
to  be  the  cause  of  the  affection.  In  some  cases  it  may  be  a  manifesta- 
tion of  hysteria,  as  instances  of  recovery  from  suggestion  prove. 

V.  Dubini's  Chorea 

By  its  manifestations  it  resembles  the  electric  chorea  of  the  preceding 
chapter,  but  by  its  course,  duration  and  termination  it  differs. 

The  onset,  which  is  abrupt  and  sudden,  is  accompanied  by  pain. 
The  latter  affects  the  head,  neck  and  lumbar  region.  The  twitchings  are 
rapid,  appear  first  in  the  upper  extremities  and  soon  spread.  Although 
they  are  rhythmical,  nevertheless  they  are  constant.  In  the  course  of 
the  disease  not  infrequently  are  observed  genuine  convulsive  seizures 
without  loss  of  consciousness.  Fever  is  also  present  in  the  majority  of 
cases. 

The  disease  is  progressive.  Gradually  the  twitchings  and  the  con- 
vulsive seizures  increase  in  intensity  and  frequency,  a  comatose  state 
supervenes  and  death  follows.  The  duration  of  the  affection  is  from  sev- 
eral days  to  four  or  five  months. 

The  sudden  onset,  the  pain,  the  accompanying  fever  and  the  associated 
pulmonary  diseases  (which  are  quite  frequent)  are  in  favor  of  an  infectious 
origin  of  the  affection.  Post-mortem  investigations  have  shown  in  a 
number  of  cases  congestion  and  inflammation  of  the  meninges,  also  of  the 
cerebral  tissue. 

TETANY 

It  is  characterized  by  bilateral,  intermittent,  painful  cramps,  especially 
in  the  hands. 

Symptoms. — The  muscular  spasms  occur  in  attacks.     The  latter  are 


520 


TETANY 


usually  preceded  by  a  few  premonitory  symptoms,  viz.  parses thesia 
(tingling,  numbness,  etc.),  general  malaise  and  sometimes  by  a  mental 
depression  and  vertigo  or  headache.  In  the  majority  of  cases  cramps 
appear  first  in  the  fingers.  The  attitude  of  the  hand  is  then  very  char- 
acteristic: it  is  either  in  a  writing  position  or  in  an  obstetrical  position, 
viz.  the  fingers  are  extended,  the  first  phalanges  are  flexed,  the  thumb  is 
against  the  palmar  surface  of  the  other  fingers,  the  entire  hand  is  flexed. 
Variations  in  this  attitude  are  observed.  When  the  interossei  and  lumbri- 
cales  are  affected,  the  hand  is  in  a  claw-like  position  (main  en  griffe.) 


Fig.  159 — A  Case  of  Tetany  During  an  Attack.     (Oppenheim.) 


When  the  contracture  spreads  and  involves  the  arm,  the  latter  is  in  a 
forced  flexion  and  applied  to  the  thorax.  If  the  lower  extremities  are 
affected,  the  flexors  of  the  foot  and  toes  are  found  mostly  in  a  state  of 
tonic  contraction.  The  toes  are  flexed  and  adducted,  the  feet  are  arched; 
the  attitude  of  the  foot  is  equino-varus. 

Tetany  may  also  affect  the  muscles  of  the  trunk,  abdomen  and  neck 
and  in  rare  cases  the  ocular  muscles.     When  the  neck  muscles  are  involved, 


TETANY  521 

the  head  is  bent  forward  and  the  chin  touches  the  chest.  Rarely  the 
dorsal  muscles  are  involved:  the  head  is  then  drawn  backward  and  the 
body  is  in  opisthotonos.  The  muscles  of  the  face,  of  the  tongue,  of  the 
ocular  muscles,  are  occasionally  affected.  The  diaphragm,  larynx,  may 
participate  and  then  the  patient  is  threatened  with  suffocation.  If  the 
sphincter  of  the  bladder  is  affected,  retention  of  urine  will  be  present. 
The  tetanic  contractions  are  usually  very  painful  and  the  least  attempt 
to  move  the  affected  parts  increases  the  pain.  Voluntary  movements  are 
impossible.  During  an  attack  the  temperature  is  slightly  elevated  and 
the  pulse  is  accelerated.  The  spasm  may  persist  during  sleep,  although 
as  a  rule  it  is  less  severe. 

In  addition  to  the  above  clinical  picture  the  following  characteristic 
symptoms  are  observed  in  tetany. 

1.  Trousseau's  Sign. — This  observer  found  that  compression  of  the 
biceps  or  immediately  below  the  inferior  insertions  of  the  deltoid  and 
in  the  lower  extremities  upon  the  internal  surface  of  the  thigh  a  tonic 
contraction  of  the  corresponding  muscles  will  be  produced.  V.  Frankl- 
Hochwart  has  shown  experimentally  that  compression  of  the  nerve-trunks 
is  the  cause  of  the  contracture.  Trousseau's  phenomenon  is  pathog- 
nomonic of  tetany. 

2.  Chvostek's  Sign. — Percussion  or  any  mechanical  irritability  of  a 
motor  or  mixed  nerve  or  the  muscles  of  the  face  produces  vivid  muscular 
contraction.  This  so-called  facial  phenomenon  is  observed  in  the  domain 
of  the  seventh  nerve.     The  symptom  is  not  constant. 

3.  Homnann's  Sign. — Pressure  upon  sensory  nerves  produces  marked 
pain  or  paresthesias.     Their  electrical  excitability  is  also  increased. 

4.  Erb's  Sign.— The  electrical  excitability  of  motor  nerves  is  increased 
so  that  a  very  mild  galvanic  or  faradic  current  gives  a  prompt  and  marked 
muscular  contraction.  The  contraction  is  not  brief  as  in  normal  con- 
dition, but  prolonged,  and  may  not  subside  until  the  current  is  inter- 
rupted. The  anodal  closure  or  opening  contracture  is  more  prompt 
than  the  cathodal  closure  contraction.  Increased  response  to  galvanism 
is  more  frequently  observed  that  to  faradism.  Erb  believes  that  not  all 
the  nerves  are  equally  apt  to  be  easily  irritated.  The  ulnar  nerve 
is  the  most  frequently  responsive. 

5.  Schlesinger's  Sign. — If  the  extended  lower  limb  is  forcibly  flexed 
over  the  pelvis,  a  spasm  will  appear  in  the  extensors  of  the  knee  and  the 
foot  is  placed  in  the  position  of  extreme  supination. 

Among  other  symptoms,  although  not  constant,  may  be  mentioned 
vaso-motor  and  trophic  disturbances,  such  as  hyperhidrosis,  herpes, 
oedema,  falling  out  of  the  nails,  muscular  atrophy.     During  the  attack 


52  2  TETANY 

the  face  may  be  flushed,  the  extremities  are  cyanosed,  the  hands  are  red. 
These  symptoms  disappear  at  the  end  of  the  attack.  The  reflexes  are 
usually  normal,  but  an  increase  or  diminution  is  sometimes  observed. 
The  objective  sensations  are  not  modified  except  when  hysteria  is  asso- 
ciated. Indicanuria  is  so  frequent  in  children  that  it  may  be  considered 
pathognomonic.     Glycosuria  has  also  been  observed. 

Occasionally  epilepsy,  exophthalmic  goiter  and  myxcedema  have  been 
found  associated  with  tetany. 

Course,  Duration,  Prognosis. — The  attacks  may  occur  every  day  or 
only  after  more  or  less  prolonged  periods:  the  intervals  may  be  hours  or 
days.  The  individual  spasms  may  last  from  a  few  minutes  to  several 
hours.  The  entire  disease  may  consist  of  but  a  few  attacks  or  else  may 
last  weeks  or  months.  Recurrences  are  not  infrequent  and  in  some  cases 
they  appear  at  regular  intervals  during  a  number  of  years.  The  prog- 
nosis in  the  majority  of  cases  is  favorable.  It  depends,  however,  upon 
the  cause.  Cases  with  gastric  dilatation  or  with  exophthalmic  goiter 
present  an  unfavorable  prognosis.  Moynihan  has  shown  that  gastric 
tetany  can  be  relieved  by  gastro-enterostomy.  When  the  respira- 
tory muscles  are  affected,  life  is  threatened.  Children  particularly  are 
in  danger  of  asphyxia  due  to  spasm  of  the  glottis. 

Diagnosis. — The  symptoms  of  tetany  are  ordinarily  so  typical  that 
the  diagnosis  is  made  without  difficulty.  In  tetanus  the  spasm  begins 
with  the  muscles  of  the  jaws  and  neck,  the  hands  and  ringers  usually 
escape,  the  temperature  is  elevated  from  the  beginning.  A  pseudo- 
tetanus,  in  which  there  are  generalized  contractures  with  albumin  in  the 
urine,  is  met  with  frequently  in  children  and  bears  an  unfavorable 
prognosis. 

In  hysteria  tonic  muscular  contractions  (pseudo -tetany)  are  unilateral, 
painless  and  not  accompanied  by  Trousseau's  and  Erb's  signs. 

Latent  cases  of  tetany  without  spasms  have  been  described.  Here 
the  only  symptoms  of  the  disease  will  be  Chvostek's  and  Erb's  signs,  also 
some  numbness  or  tingling  of  the  hands. 

Etiology. — Certain  occupations  play  a  predisposing  role.  According 
to  Frankl-Hochwart's  statistics,  among  314  patients  141  were  shoemakers 
and  42  tailors.  Certain  countries  are  more  affected  than  others.  The 
disease  is  frequent  in  Sweden,  Austria  and  Germany. 

Epidemics  of  the  affection  have  been  observed.  Men  between  fifteen 
and  twenty-five  are  particularly  attacked.  In  women  the  disease  occurs 
during  pregnancy  or  lactation  and  puerperal  state. 

Infection  and  intoxication  play  a  predominant  part  in  the  causation 
of  tetany.     In  the  course  of  infectious  diseases,  such  as  typhoid  fever, 


TETANY  523 

grippe,  scarlet  fever,  measles,  malaria,  or  during  convalescence  tetany 
may  occur.  Among  toxic  conditions  gastro-intestinal  disorders  are  very 
frequently  the  cause  of  tetany.  Gastro-enteritis  is  almost  the  only  cause 
of  tetany  in  children.  Tetany  has  also  been  seen  in  association  with 
albuminuria.  Extirpation  of  the  thyroid  gland  has  been  followed  by 
tetany.  Here  also  a  toxic  element  is  the  cause  of  the  disease.  It  is  prob- 
able that  in  such  cases  the  parathyroids  were  removed  together  with  the 
thyroid  gland.  Removal  of  the  parathyroids  has  been  seen  to  be  followed 
by  tetany. 

Rickets,  osteomalacia,  intestinal  worms,  use  of  alcohol,  ergot,  inhala- 
tion of  alcohol,  may  be  also  accompanied  by  tetany. 

Local  irritation,  as  the  use  of  a  stomach  pump  or  passing  of  a  sound, 
percussion  of  the  region  of  the  stomach,  cold,  emotion,  exertion,  are  the 
exciting  causes. 

Pathogenesis. — No  reliance  can  be  placed  upon  the  findings  at  the 
autopsies.  The  results  are  contradictory  and  in  the  majority  of  cases 
negative.  Mention  can  be  made  of  some  poliomyelitic  changes  in  the 
cells  of  the  anterior  cornua  of  the  spinal  cord.  The  consensus  of  opinion 
is  that  in  the  majority  of  cases  the  disease  is  due  to  a  toxic  or  infectious 
condition.  The  occurrence  of  it  in  connection  with  gastro-intestinal 
disorders,  with  removal  of  the  thyroid  or  parathyroid  glands,  with  in- 
fectious diseases,  speaks  in  favor  of  the  above  view.  Experimental  re- 
searches and  close  observations  on  operative  cases  are  strongly  in  favor  of 
parathyroid  insufficiency  as  being  the  pathogenetic  factor  in  tetany. 
But  what  toxic  element  is  at  work,  it  is  at  present  difficult  to  ascertain. 

Gastric  Tetany. — In  this  form  of  tetany  an  ulcer  of  the  stomach 
with  resulting  pyloric  stenosis  and  gastric  dilatation  have  been  found. 
There  is  usually  hyperchlorhydria.  The  typical  symptoms  of  tetany 
are  present.  The  intensity  of  the  spasms  may  vary  from  case  to  case. 
Sometimes  they  are  so  slight  or  occur  so  rarely  that  tetany  may  be 
overlooked.  In  the  majority  of  cases  the  spasms  are  severe.  The  only 
radical  measure  in  such  cases  is  gastro-enterostomy.  As  to  the  patho- 
genesis of  gastric  tetany,  it  may  be  presumed  that  a  toxic  element 
from  the  stomach  is  added  to  the  already  existing  parathyroid  insuffi- 
ciency. Langmead  has  recently  called  attention  to  tetany  in  association 
with  dilatation  of  the  colon.  The  spasms  occur  regularly  and  appear  to 
be  in  intimate  relation  with  the  character  of  the  feces.  The  condition 
is  met  with  in  children  of  arrested  physical  and  intellectual  development. 

Treatment. — Removal  of  the  cause  is  the  first  indication.  Gastro- 
intestinal disorders  should  be  remedied  by  intestinal  antiseptics,  enemas 
and  emetics  if  necessary,  but  the  stomach-pump  must  be  avoided  (see 


524  TETANY 

reasons  above).  In  cases  of  operations  on  goiter  care  must  be  taken  to 
avoid  total  thyroidectomy.  When  the  latter  is  unavoidable,  thyroid 
or  parathyroid  extract  must  be  administered  internally. 

When  exposure  to  cold  is  the  cause,  warm  baths  and  diaphoretics  are 
of  benefit.  The  spasms  can  be  relieved  by  sedative  medications,  such  as 
bromides,  morphine,  chloral.  Rest  in  bed  is  an  excellent  measure  in 
some  cases.  Trousseau  advises  application  of  ice  to  the  spine.  On  the 
other  hand,  tepid  baths  administered  several  times  a  day  for  ten  to  fifteen 
minutes  may  be  of  great  benefit.  Galvanism  may  sometimes  render  good 
services.  In  one  of  my  cases  absolute  rest  with  milk  diet  gave  me  very 
satisfactory  results.  In  the  gastric  form  of  the  affection,  which  is  rebellious 
to  treatment,  gastro-enterostomy  should  be  undertaken  without  delay,  as 
excellent  results  have  been  reported.  In  cases  of  asphyxia  due  to  spasm 
of  the  glottis,  hypodermic  injections  of  pilocarpine  or  application  of 
a  wet  cloth  to  the  neck  may  be  useful.     Digitalis  is  advised  by  Gowers. 

The  researches  of  Loeb  and  J.  B.  MacCallum  show  that  there  is  a 
great  relationship  between  a  tetany  and  reduction  of  calcium  salts  in  the 
organism.  An  analysis  of  blood  taken  from  a  dog  during  tetany  shows  an 
amount  of  calcium  which  is  only  about  a  half  that  of  a  normal  dog  on  the 
same  constant  diet.  It  is  also  known  that  the  parathyroids  control  the 
calcium  metabolism,  so  that  upon  their  removal  a  rapid  excretion  deprives 
the  tissues  of  calcium  salts.  For  these  reasons  administration  of  calcium 
salts  or  of  parathyroids  is  indicated  in  tetany.  The  intravenous  method 
is  the  best  for  administration  of  calcium  salts.  Forty  to  80  grains  of 
calcium  lactate  are  diluted  in  400-500  c.c.  of  normal  salt  solution.  The 
injection  can  be  repeated  in  twenty-four  hours  if  necessary.  The  para- 
thyroids may  be  administered  by  mouth,  intravenously  or  by  grafting. 
The  intravenous  method  gave  the  best  results.  Krabbel  {Beitr.  z.  klin. 
Chir.,  191 1)  has  recently  reported  excellent  results  from  implanting  para- 
thyroid bodies  in  the  tibia  of  one  patient  and  in  the  preperitoneal  space 
of  another  patient. 

MYOSPASM  FROM  INTENSE  HEAT 

This  condition  was  first  described  in  1904  by  Edsall  (Amer.  J.  Med. 
Sciences)  and  later  from  a  larger  number  of  cases  by  Cameron  (/.  Am.  Med. 
Ass.,  1909). 

Symptoms. — In  working  men  exposed  to  intense  heat  (i4o°-235°  F.) 
such  as  iron- workers  or  men  employed  in  firerooms  of  vessels,  a  very  painful 
tonic  spasm  of  the  muscles  develops  spontaneously  or  upon  the  least  volun- 
tary effort.     An  attack  lasts  from  half  a  minute  to  a  minute  and  occurs 


THOMSEN;S    DISEASE  525 

very  frequently  during  the  illness,  the  duration  of  which  is  about  twenty- 
four  hours.  A  sense  of  exhaustion  and  soreness  with  tingling  in  the  muscles 
remains  for  some  time.  Between  the  individual  spasms  a  fibrillary  con- 
traction of  the  affected  muscles  is  distinctly  noticeable.  The  muscles  of 
the  forearms  and  legs,  also  the  abdominal  muscles,  are  usually  involved. 
The  condition  grossly  resembles  tetany,  but  the  special  symptoms  char- 
acteristic of  the  latter  are  absent,  viz.  Erb's,  Trousseau's  and  Hoffmann's 
sign.  The  mechanical  irritability  of  the  muscles  is  increased.  The  re- 
flexes are  normal,  there  are  no  changes  in  the  general  objective  sensi- 
bility (touch,  pain  and  temperature),  in  the  sphincters  and  in  the  pupils. 

Etiology. — The  spasms  are  due  to  the  intense  heat  of  the  atmosphere 
in  which  the  work  is  done.  They  occur,  according  to  Cameron,  usually 
after  the  men  have  been  working  for  some  time.  Men  on  the  day  turn 
would  be  attacked  toward  the  close  of  the  morning's  or  afternoon's  work, 
and  the  night-turn  men  toward  midnight.  Overwork  is  probably  one 
of  the  predisposing  factors.  Alcoholism  is  probably  another  predisposing 
cause.  Cameron  observed  that  the  spasms  occurred  mostly  in  hard 
drinkers.  Lowered  muscular  tone  from  other  causes  is  also  one  of  the 
predisposing  elements. 

Prognosis. — One  attack  usually  predisposes  to  others.  Recovery  is 
possible.  However,  fatal  cases  have  been  reported.  They  may  be  due 
to  a  spasm  of  heart  muscle. 

Pathogenesis.- — Nothing  definite  is  known  as  to  the  nature  of  the 
disorder.  Disturbances  of  metabolism  producing  some  degenerative  con- 
dition of  the  muscles  have  been  suggested. 

Treatment. — It  must  naturally  be  symptomatic.  Pain  may  be  re- 
lieved by  the  usual  remedies  or  by  a  general  anaesthetic.  A  mild  inter- 
rupted faradic  current  gave  Cameron. some  satisfactory  results. 

THOMSEN'S  DISEASE 

(Myotonia  Congenita) 

Symptoms. — The  chief  symptom  is  a  sudden  tonic  contraction  of  a 
group  of  muscles  when  an  attempt  is  made  to  make  a  forced  movement. 
At  first  there  is  an  inability  to  continue  the  movement,  but  gradually 
and  very  slowly  the  muscles  relax  and  the  affected  limb  is  able  to  perform 
the  act.  The  stronger  the  contraction  is  the  longer  is  the  relaxation. 
When  later  an  attempt  is  made  to  stop  or  to  modify  the  movement,  a 
new  spasm  occurs.  As  the  lower  extremities  are  the  usual  seat  of  myotonia, 
it  is  in  the  act  of  walking  that  the  phenomenon  is  mostly  observable.  All 
the  muscles  may  become  affected,  especially  those  of  the  extremities,  but 


526  thomsen's  disease 

those  of  the  trunk  and  neck  are  less  frequently  involved  than  those  of  the 
extremities.  Exceptionally  the  muscles  of  the  tongue  and  the  masti- 
catory muscles  are  involved.  The  muscles  of  deglutition  and  ocular 
muscles  are  very  rarely  affected. 

The  myotonic  spasm  is  increased  by  reflex  acts,  such  as  sneezing, 
coughing,  etc.,  by  cold,  exertion  and  especially  by  an  emotion.  On  the 
contrary  it  is  decreased  by  heat,  physical  and  mental  rest. 

The  muscles  affected  by  myotonia  are  generally  hypertrophied,  but 
their  power  is  diminished.  The  following  special  signs  are  characteristic 
of  the  myotonic  muscles  and  of  the  nerves  distributed  in  them. 

Erb  called  attention  to  the  fact  that  mechanical  irritability  of  the 
nerves  is  normal  or  diminished.  Their  electrical  reactions  are  as  follows. 
The  faradic  and  galvanic  irritability  of  the  nerves  are  quantitatively 
normal  when  a  moderate  degree  of  stimulation  is  applied.  Should  the 
stimulation  be  prolonged,  a  persistent  tonic  contraction  will  follow. 
Mechanical  irritability  of  the  muscles  is  increased.  The  electrical 
contractility  presents  here  a  special  feature. 

With  the  galvanic  current,  the  KCC  and  AnCC  are  equal.  The  con- 
tractions are  sluggish  and  continue  long  after  the  excitation  has  ceased. 
With  the  faradic  current  the  contraction  also  lasts  a  long  time  (about  a 
half  of  a  minute)  and  undulation  of  the  muscle  is  observed.  These  phe- 
nomena constitute  the  so-called  "myotonic  reaction." 

Psychic  disturbances  and  epilepsy  are  sometimes  associated  with 
Thomsen's  disease.  Muscular  atrophy  and  multiple  neuritis  have  also 
been  reported  in  connection  with  myotonia  congenita. 

Course,  Duration,  Prognosis. — The  disease  makes  its  first  appear- 
ance in  infancy,  but  becomes  well  developed  at  the  age  of  twenty.  Exer- 
tion aggravates  it.  It  may  be  arrested,  but  frequently  recurs.  Life  is 
not  threatened,  but  the  affection  is  incurable;  it  lasts  all  life. 

Diagnosis. — The  special  characteristics  of  the  disease  are  sufficient 
for  diagnosis.  Eulenburg  described  a  "paramyotonia  congenita,"  which 
is  recognized  by  a  symmetrical  muscular  rigidity  without  Erb's  myotonic 
reaction;  it  usually  follows  exposure  to  cold. 

Etiology. — Heredity  plays  a  predominant  part.  Thomsen  traced 
over  twenty  members  in  his  own  family.  Males  are  more  frequently 
affected  than  females. 

Pathogenesis. — The  consensus  of  opinion  is  that  the  disease  is  a  form 
of  myopathy  (see  this  chapter).  Histological  studies  show  hypertrophy 
of  muscular  fibers  and  proliferation  of  their  nuclei.  The  central  nervous 
system  is  intact.  Congenital  abnormal  development  is  probably  the  true 
nature  of  the  malady. 


OCCUPATION    NEUROSES  527 

Treatment. — All  the  factors  that  are  apt  to  increase  the  muscular 
tonicity  (see  above)  should  be  avoided.  Moderate  exercises  and  rational 
gymnastics  may  be  beneficial. 

OCCUPATION  NEUROSES 

(Occupation  Spasms) 

Under  this  name  is  understood  a  motor  disturbance  consisting  of  a 
sudden  cramp  in  a  group  of  muscles  used  in  certain  acts  and  brought  on 
exclusively  during  the  execution  of  those  acts. 

This  functional  disturbance  may  occur  in  any  portion  of  the  body,  but 
more  particularly  in  the  upper  extremities.  The  most  typical  form  of 
these  neuroses  is 

Writer's  Cramp 

It  develops  slowly.  At  first  the  patient  notices  a  certain  fatigue  and 
stiffness  in  the  fingers  while  writing,  so  that  he  is  obliged  to  rest  for  a  while. 
Soon  he  finds  that  the  interval  of  rest  must  be  increased;  the  difficulty  of 
writing  appears  as  soon  as  the  act  is  commenced.  He  is  forced  to  have 
recourse  to  various  positions,  use  both  hands,  etc.,  and  finally  give  up 
completely  the  act  of  writing. 

The  phenomenon  may  present  itself  in  the  spastic,  paralytic  and 
tremulous  forms. 

Spastic. — A  sudden  extension  of  the  index  -and  flexion  or  adduction 
of  the  thumb  or  vice  versa  is  the  initial  manifestation.  Sometimes  the 
medius  and  the  other  fingers  suddenly  flex.  In  some  cases  the  condition 
extends  to  the  forearm,  arm  and  shoulder.  This  is  due  to  the  fact  that  in 
the  act  of  writing  participate  not  only  the  muscles  of  the  hand,  but  also 
those  of  the  forearm  and  arm. 

The  cramp  will  persist  as  long  as  the  act  of  writing  is  insisted  upon. 
In  advanced  cases  pain  is  present,  but  only  when  an  attempt  is  made  to 
write. 

The  characteristic  feature  of  the  condition  is  the  appearance  of  a 
spasm  in  the  muscles  of  the  hand  only  in  the  act  of  writing,  but  absence  in 
any  other  act  executed  by  the  same  muscles.  However  in  cases  of  long 
duration  any  fine  and  delicate  act  of  the  fingers  may  bring  on  an  attack. 

Paralytic  Form.— It  consists  of  a  sudden  sensation  of  fatigue  and  numb- 
ness in  the  hand  while  writing.  The  hand  remains  applied  to  the  paper. 
As  soon  as  the  penholder  is  removed,  the  sensation  disappears.  A  true 
paralysis  of  the  adductors  of  the  thumb  has  been  observed. 


528  OCCUPATION   NEUROSES 

Tremulous  Form. — Instead  of  a  spasm  or  paresis  there  may  be  only 
a  tremor  in  the  fingers  or  in  the  entire  arm  during  the  act  of  writing. 

The  course  of  writer's  cramp  is  usually  chronic.  Periods  of  ameliora- 
tion and  aggravation  are  observed.  It  may  last  an  indefinite  time. 
Recurrences  are  frequent.     Recoveries  are  possible. 

Other  Occupation  Spasms 

Pianist's  Cramp. — It  is  mostly  of  the  paralytic  form.  It  manifests 
itself  as  a  sudden  functional  inability  to  continue  playing.  The  right  hand 
is  more  frequently  affected  than  the  left,  although  both  hands  may  become 
involved. 

Violinist's  Cramp  may  present  itself  in  the  paralytic  and  spastic  forms 
and  mostly  in  the  left  hand. 

Telegraphist's  Cramp  presents  the  same  course  and  the  same  varieties 
as  writer's  cramp.  In  one  of  my  patients  there  was  also  pain  along  the 
nerve-trunks  of  the  right  arm. 

Shoemaker's,  Tailor's,  Seamstress's  Cramp  occurs  in  the  muscles 
which  are  exercised  in  the  act  of  sewing  or  cutting  with  large  scissors. 

In  blacksmiths  the  cramp  is  localized  in  the  biceps  and  deltoid 
muscles. 

The  lower  extremities  are  rarely  affected. 

Dancers'  Cramp  occurs  usually  in  the  thigh  muscles. 

The  face  is  also  rarely  involved.  In  watchmakers  and  trumpet- 
players  a  spasm  of  orbicularis  palpebrarum  has  been  observed. 

Pathogenesis. — Various  opinions  have  been  expressed  concerning  the 
nature  of  occupation  neuroses.  Some  believe  in  a  muscular  origin,  others 
that  the  condition  is  due  to  an  irritation  of  the  peripheral  nerves  and  still 
others  in  a  central  cause. 

The  fact  that  a  muscle  or  a  group  of  muscles  will  be  thrown  into  a 
state  of  spasm  only  when  they  are  called  upon  to  execute  a  certain  given 
act  and  contract  normally  in  other  acts  speaks  against  the  muscular  theory 
( muscular  traumatism  and  myositis).  On  the  other  hand,  there  are  many 
individuals  who  write  daily  a  great  deal,  pianists  who  play  long  hours,  and 
still  do  not  become  affected  with  writer's  or  pianist's  cramp.  It  is  evident 
that  only  a  certain  class  of  persons  is  subject  to  this  nervous  disorder.  The 
latter  is  met  with  in  predisposed  or  neuropathic  individuals  and  the  dis- 
turbance is  dependent  upon  a  mental  cause.  It  is  sometimes  associated 
with  other  mental  disturbances,  as  phobias,  abulias  (see  Neurasthenic 
Insanities).  Not  infrequently  it  is  observed  in  several  members  of  the 
same  family.     Some  writers  believe  with  Duchenne  that  there  is  a  disturb- 


PARALYSIS    AGITANS  529 

ance  in  the  center  of  coordination.     Others  think  that  the  cramp  is  due 
to  a  neuralgia  caused  by  molecular  changes  in  the  sensory  fibers. 

Treatment. — Abstention  from  work  which  causes  the  cramp  is  the 
first  indication.  General  hygienic  measures  with  massage  and  hydro- 
therapy are  beneficial  in  view  of  the  neuropathic  make-up  of  the  patients. 
Local  massage  may  be  of  use.  Electricity  has  given  no  appreciable 
results.  No  special  internal  medication  is  to  be  mentioned.  I  obtained 
very  satisfactory  and  sometimes  perfect  results  from  Bier's  method.  In 
a  series  of  cases  (published  in  the  Therapeutic  Gazette,  1908)  I  applied  this 
treatment  systematically  in  every  case  to  the  exclusion  of  all  other  treat- 
ment and  the  patients  were  allowed  to  continue  their  work  moderately. 
I  found  that  in  some  cases  a  few  applications  of  the  bandage  above  the 
elbow — for  an  hour  twice  a  day — gave  the  patients  great  relief.  Complete 
recovery  also  followed  in  some  cases.  It  is  apparent  that  the  circulation  in 
the  affected  muscles  has  somewhat  to  do  with  the  cramp.  Reeducation 
is  a  good  method.  The  patient  is  advised  to  practice  his  usual  exercises 
for  a  very  brief  time  every  day  and  gradually  increase  the  duration  and  the 
number  of  the  seances.  Benedikt  {Wiener  klin.  Wochenschr.,  191 1)  ob- 
tained very  satisfactory  results  from  injections  of  2  per  cent,  solution  of 
phenol.  He  believes  that  the  original  source  of  occupation  neurosis  is  in 
the  tendons  and  muscles.  Tender  points  can  always  be  found  in  the 
tendons  of  the  wrist  and  hand  or  in  the  heads  of  the  tendons  in  the 
elbow  or  shoulder.  Injection  of  weak  phenol  solution  at  these  tender 
points  restores  the  condition  to  normal.  Sometimes  several  injections 
are  necessary. 

PARALYSIS  AGITANS  (PARKINSON'S  DISEASE) 

Shaking  Palsy 

Symptoms.— Tremor,  attitude,  gait,  fades,  are  the  elements  presenting 
special  features  in  the  disease. 

Tremor. — It  is  present  in  the  majority  of  cases.  It  is  passive  in 
character,  viz.  it  is  present  when  the  body  is  at  rest.  It  usually  disap- 
pears upon  voluntary  movements,  but  returns  if  the  latter  are  sustained. 

The  tremor  may  affect  the  entire  body,  but  more  frequently  the  upper 
extremities  and  particularly  the  hands  and  fingers.  Sometimes  all  the 
ringers  are  agitated,  but  the  thumb  is  especially  affected.  It  moves  to  and 
fro  over  the  palmar  surface  of  the  other  fingers  in  a  continuous  and  slow 
manner;  its  oscillations  remind  the  act  of  rolling  pills  or  crumbling  bread. 
The  tremor  is  rhytnmical. 

The  tremor  decreases  from  the  distal  end  toward  the  root  of  the  limb, 
so  that  it  is  not  perceptible  at  the  shoulder. 

34 


53° 


PARALYSIS    AGITANS 


In  the  lower  extremities  the  foot  is  particularly  affected.  When  the 
patient  is  seated,  the  toes  are  held  against  the  floor,  but  the  heel  keeps  on 
striking  the  floor  in  a  continuous  and  rhythmical  manner. 

The  tremor  of  the  head  is  usually  transmitted  by  the  arms.  When 
there  is  a  primary  tremor  of  the  head,  it  is  the  result  of  a  contraction  of 
the  neck  muscles.  The  tremor  is  then  perceptible  on  the  lips.  The 
patient  gives  then  the  impression  of  muttering  silently. 

The  tremor  of  the  extremities  as  a  rule  does  not  interfere  with  ordinary 

active  movements,  provided  the  latter 
are  not  prolonged.  Writing  is  difficult. 
The  tremor  disappears  during  sleep. 
Emotions  and  exertion  increase  it.  A 
continuous  vibration  of  the  body  occur- 
ring, for  example,  in  traveling  de- 
creases the  intensity  of  the  tremor  and 
renders  the  patient  more  comfortable. 

Attitude. — In  a  typical  case  the  pa- 
tient's head  is  inclined  forward  and  as 
if  fixed  to  the  trunk,  the  back  is  curved 
(kyphosis).  He  holds  himself  rigid, 
turns,  walks,  sits  down,  gets  off  his  chair 
as  one  rigid  mass  (rod-like) .  This  con- 
dition is  due  to  the  muscular  rigidity 
which  is  so  characteristic  of  Parkinson's 
disease.  Upon  passive  movements 
considerable  resistance  is  felt.  Al- 
though in  the  majority  of  cases  the  body  is  in  a  state  of  semiflexion, 
there  are  also  cases  of  extension  type;  the  head  is  held  backward,  and 
instead  of  kyphosis  there  is  lordosis  of  the  spine.  The  movements  of  the 
body  are  naturally  slow  and  monotonous. 

It  is  well  to  remember  that  the  muscular  rigidity  is  not  that  of  organic 
nervous  nature,  as  for  example  is  spastic  paraplegia  or  hemiplegia.  It  is  a 
rigidity  of  a  cadaver,  as  Bloch  has  well  said.  The  rigidity  produces  a 
certain  degree  of  muscular  weakness,  which  disables  the  patient  for  work  or 
even  for  ordinary  voluntary  acts.  A  true  paresis  may  occur  only  in 
advanced  stages.  The  patient  is  in  a  state  of  restlessness;  there  is  a 
tendency  to  change  position. 

Gait. — In  mild  cases  the  only  peculiarities  noticeable  are  small  quick 
steps  and  slow  gait.  In  advanced  cases  the  following  is  observed.  When 
the  patient  attempts  to  walk,  he  inclines  the  body  forward,  steps  first  on 
his  toes  and  then  for  fear  of  falling  he  is  obliged  to  accelerate  his  gait  and 


Fig.  160. 


PARALYSIS    AGITANS  53 1 

run.  In  some  cases  there  is  only  an  accelerated  gait  (f estination) ,  in 
others  a  distinct  tendency  to  fall  forward  (propulsion).  The  latter  is 
easily  noticed  when  a  slight  push  is  given  on  the  back.  The  patient  will 
keep  on  running  until  an  obstacle  is  met.  The  same  phenomenon  is 
observed  when  the  patient  is  pushed  backward  (retropulsion)  or  laterally 
(lateropulsion). 

Facies. — It  is  typical.  It  is  characterized  by  immobility  of  features. 
It  is  mask-like.  It  gives  the  impression  of  astonishment,  surprise,  fright. 
This  expression  is  independent  of  the  inner  feelings  of  the  patient.  The 
conditions  of  the  facies  as  well  as  the  fixed  expression  of  the  eyes  are  due 
to  the  rigidity  of  the  facial  and  ocular  muscles,  respectively. 

Other  Symptoms.— The  speech  is  not  infrequently  changed.  Mo- 
notonous voice  and  rapidity  of  words  are  its  characteristics. 

Sensory  disturbances  are  only  of  a  subjective  nature.  The  patients 
frequently  complain  of  rheumatic  pain  in  the  limbs,  of  muscular  fatigue 
and  numbness.  Sensation  of  heat  is  continuously  present  and  sometimes 
accompanied  by  abundant  perspiration.  The  tendon  reflexes  are  either 
normal  or  diminished  or  more  frequently  exaggerated.  Ankle-clonus, 
Babinski  and  paradoxical  phenomena  are  absent. 

Trophic  disturbances  are  usually  absent.  In  some  cases  there  is  a 
thickening  and  hardening  of  the  skin,  especially  on  the  face.  Rheumatoid 
deformities  of  hands  and  feet  are  also  observed.  Muscular  atrophy  occurs 
in  the  last  stages  of  the  disease. 

Vaso-motor  Disturbances  have  been  mentioned  above.  They  consist 
of  a  feeling  of  heat  and  hyperhidrosis.  Cyanosis  of  the  extremities,  also 
localized  oedemata,  may  occur. 

The  mentality  is  usually  intact.  However  there  is  a  certain  degree  of 
indifference,  apathy  and  even  depression.  There  is  an  intellectual  fixation 
alongside  the  physical  fixation.  This  mental  condition  varies  from  time 
to  time  in  the  same  individual.  In  some  cases  there  may  be  mental  failure 
and  even  a  certain  degree  of  dementia. 

Course,  Duration,  Prognosis. — The  course  of  paralysis  agitans  is  slow. 
In  some  cases  it  develops  suddenly,  as  for  example  in  cases  following  a 
trauma  or  emotion.  In  the  majority  of  cases  it  commences  with  a  tremor 
in  one  extremity,  most  frequently  in  one  of  the  upper  extremities.  Gradu- 
ally the  lower  extremity  of  the  same  side  becomes  affected.  Later  the 
opposite  side  is  invaded.  The  development  of  the  symptoms  is  slow,  but 
progressive.  Several  years  elapse  before  the  disease  is  generalized. 
Remissions  with  amelioration  of  the  symptoms  may  occur,  but  no  com- 
plete disappearance  of  the  latter. 

The  disease  may  last  many  years,  from  ten  to  thirty.     Death  usually 


532  PARALYSIS    AGITANS 

intervenes  from  some  intercurrent  disease,  particularly  from  pneumonia. 
If  this  is  not  the  case,  the  disease  progresses  until  complete  physical  dis- 
ability occurs.  The  patient  is  then  confined  to  bed;  bed-sores  and  cachexia 
hasten  death. 

Forms. — The  above  clinical  picture  presents  the  most  frequent  variety 
of  paralysis  agitans.  There  is  also  a  form,  in  which  the  tremor  is  absent 
and  the  disease  is  then  recognized  by  the  rigidity,  f acies  and  attitude.  In 
another  variety  the  tremor  alone  is  present  or  else  the  rigidity  is  extremely 
slight.  In  some  cases  the  tremor  is  not  only  passive,  but  also  intentional 
(similar  to  that  of  multiple  sclerosis).  There  are  also  cases  in  which 
instead  of  flexion  of  the  trunk  and  extremities  there  is  extension. 

Diagnosis. — The  symptoms  as  a  rule  are  so  typical  that  an  error  in 
diagnosis  is  rare.  The  variety  without  tremor  may  present  some  difficul- 
ties. 

The  tremor  may  lead  sometimes  to  an  erroneous  diagnosis,  especially 
when  besides  being  passive  it  is  also  intentional.  Multiple  sclerosis 
should  be  then  thought  of.  But  in  the  latter  disease  there  are  also  nystag- 
mus, scanning  speech  and  absence  of  passive  tremor. 

In  paresis  the  tremor  is  also  intentional,  but  there  are  also  changes  in 
speech,  in  the  pupillary  reflexes;  finally  mental  symptoms. 

A  senile  tremor  is  usually  passive  and  intentional,  but  it  is  not  asso- 
ciated with  other  symptoms  characteristic  of  paralysis  agitans.  Besides, 
in  the  senile  form  the  head  is  generally  affected  early. 

Hysteria  may  simulate  paralysis  agitans  by  its  tremor.  In  one  of  my 
cases  it  was  limited  to  one  hand,  but  the  movements  were  somewhat  of 
wider  range,  they  were  present  when  the  arm  was  at  rest  and  upon  volun- 
tary movements;  the  characteristic  attitude,  f acies  and  gait  of  Parkinson's 
disease  were  absent. 

Organic  hemiplegia  may  sometimes  be  accompanied  by  a  tremor  on 
the  same  side  and  thus  simulate  a  form  of  paralysis  agitans  in  which  the 
tremor  is  confined  to  one  hand.  In  the  majority  of  such  cases  the  diagnosis 
will  not  be  difficult  in  view  of  the  characteristic  symptoms  of  both  affec- 
tions, but  there  are  cases  which  present  almost  insurmountable  difficulties 
in  differentiating  them. 

Pathogenesis. — The  post-mortem  findings  present  nothing  definite 
as  to  the  nature  of  the  disease.  While  some  autopsies  are  negative,  others 
show  perivascular  sclerosis  in  the  gray  and  white  matter  of  the  brain  or 
spinal  cord.  The  most  recent  work  on  the  subject  is  that  of  G.  Maillard, 
who  considers  the  disease  as  due  to  arteriosclerotic  changes  in  the  red 
nucleus.  On  the  contrary  a  great  many  observers  found  changes  only  in 
the  muscles,  viz.   nuclear  proliferation  in  the  sarcolemma,    atrophy    of 


AKINESIA    ALGERA  533 

some  fibers,  diminution  of  muscle-spindle,  and  they  place  the  disease 
among  myopathies  (see  this  chapter).  In  the  peripheral  nerves  increase 
of  interstitial  tissue  and  slight  degenerative  changes  in  the  fibers  have  been 
found  in  some  cases. 

Some  writers  believe  that  the  disease  is  due  to  some  toxic  agent  pro- 
duced by  the  parathyroid  bodies  (Lundborg,  Berkeley  and  others).  They 
base  their  opinion  on  this  observation  that  the  symptoms  following 
parathyroidectomy  resemble  much  those  of  paralysis  agitans.  Until  a 
solid  anatomical  basis  is  found  paralysis  agitans  should  be  considered  as  a 
functional  nervous  disease.     This  is  the  opinion  of  the  majority  of  authors. 

Etiology. — The  exciting  causes  are  traumatism  and  emotions  of  a 
depressive  character. 

A  neuropathic  tendency  plays  an  important  predisposing  part. 

The  disease  affects  both  sexes,  but  men  more  frequently  than  women. 
The  usual  age  at  which  it  occurs  is  beween  forty  and  sixty,  although  it 
may  develop  before  twenty,  as  some  records  show. 

Treatment. — The  tremor  is  sometimes  ameliorated  by  trepidation  in  a 
carriage  or  train.  One  of  my  patients  obtained  great  relief  by  riding  on 
a  train  two  hours  every  day.  For  the  same  reason  I  treated  another  of 
my  patients  with  a  very  frequently  interrupted  faradic  current  and 
succeeded  in  diminishing  the  intensity  of  the  tremor. 

Internally  hyoscine  hydrobromate  in  gr.  i/ioo  doses  two  or  three  times 
a  day  relieves  sometimes  the  tremor  as  well  as  the  rigidity.  Among  other 
drugs  may  be  mentioned:  cannabis  indica,  codein,  opium,  arsenic,  bro- 
mides, veratrum  viride. 

Warm  Baths  are  useful  to  control  the  rigidity.  Massage  gently 
applied  may  do  some  good.  Systematic  exercises  sometimes  give  satis- 
factory results  in  decreasing  the  rigidity. 

Rest,  which  is  so  beneficial  in  other  neuroses,  is  contraindicated  here. 
However  violent  exercises  or  undue  fatigue  must  be  avoided. 

According  to  the  latest  researches,  particularly  of  Loeb  and  J.  B. 
MacCallum,  also  of  W.  G.  MacCallum  and  C.  Voegtlin,  there  is  a  relation- 
ship between  various  twitchings  and  calcium  metabolism  and  the  function 
of  the  parathyroid  glands.  There  is  consequently  a  therapeutic  indi- 
cation for  the  use  of  calcium  salts  and  of  parathyroids  in  paralysis  agitans. 

AKINESIA  ALGERA 

Under  this  name  Moebius  in  1891  described  a  symptom-group  char- 
acterized by  an  inability  to  move  about  because  of  pain.  If  some  slight 
movements  are  possible,  the  condition  is  called  "dyskinesia  algera." 


534  AKINESIA   ALGERA 

At  first  only  forced  movements  are  painful,  but  gradually  the  most 
insignificant  displacements  become  intolerable.  The  pain  appears  upon 
voluntary  movements,  persists  after  the  movements  ceased.  Little  by 
little  any  movement  becomes  impossible.  There  is,  however,  no  genuine 
paralysis. 

Pain  is  present  not  only  in  the  muscles  executing  movements,  but  it 
extends  to  the  remotest  parts  of  the  body.  Thus  headache  may  develop. 
The  latter  may  appear  upon  the  least  mental  exertion.  Sometimes 
mental  fatigue  provokes  pain  in  the  entire  body,  especially  in  those 
muscles  that  are  called  upon  to  contract. 

In  some  cases  a  light  causes  pain  (dysopsia  algera).  In  Oppenheim's 
patient  the  sight  of  white  objects  caused  pain.  In  analog}'  with  painful 
motions  the  light  phenomenon  can  be  called  "painful  photophobia." 
It  is  particularly  noticeable  upon  attempts  of  reading. 

Some  patients  feel  a  fatigue  in  the  head  and  severe  headache  upon 
the  least  effort  to  read,  write  or  speak.  It  is  the  so-called  apraxia  algera. 
In  Erb's  case  hearing  caused  pain,  so  that  the  patient  could  not  converse. 
In  another  of  Oppenheim's  patients  ingestion  of  food  awakened  pain, 
while  there  was  no  disease  of  the  stomach. 

Xeftel  described  an  analogous  form  under  the  name  of  "atremia," 
where  the  patient  while  lying  can  move  around  without  pain,  but  as  soon 
as  he  attempts  to  get  up,  he  suffers  in  the  back  and  head. 

Course,  Duration,  Prognosis. — In  the  majority  of  cases  the  disease 
is  rebellious  to  treatment  and  may  last  an  indefinite  time.  Ameliorations 
occur,  but  recurrences  are  frequent.  Complete  recovery  is  also  possible. 
Mental  disturbances,  as  delusions  and  hallucinations,  not  infrequently 
develop  in  the  course  of  the  disease.  Some  patients  die  in  a  fully  developed 
psychosis.     The  prognosis  is  bad  in  the  majority  of  cases. 

Etiology  and  Pathogenesis. — Functional  nervous  diseases  (neuras- 
thenia, hysteria,  hypochondria)  play  a  very  important  predisposing  role. 
Their  association  with  akinesia  algera  is  quite  frequent. 

The  consensus  of  opinion  is  that  the  condition  is  a  neurosis  and  should 
be  placed  alongside  other  well-known  functional  nervous  diseases,  that 
the  pain  is  central  with  complete  integrity  of  the  nervous  tissue.  It  is  a 
fixed  sensation,  a  disease  of  attention,  a  painful  hallucinatory  obsession. 

Treatment. — Suggestion  and  autosuggestion  are  the  main  elements 
of  treatment.  What  a  patient  can  accomplish  by  exercising  his  will 
power  and  with  a  continuous  effort  to  help  himself  can  be  seen  from  Erb's 
case,  in  which  the  patient  after  remaining  in  bed  nineteen  years  finally 
learned  how  to  convince  himself  that  his  trouble  was  not  real,  that  it  was 
only  hallucinatory.     The  patient  improved  considerably. 


HEADACHE  535 

Reeducation  of  movements  with  a  great  deal  of  persistence  and  per- 
suasion tactfully  carried  out,  aided  by  gentle  massage,  can  in  some  cases 
accomplish  much  good.  Oppenheim's  patient  mentioned  above  recovered 
by  wearing  blue  glasses,  also  by  taking  arsenic. 

HEADACHE 

Cephalalgia 

Headache  is  not  a  disease  but  a  nervous  symptom  accompanying 
various  diseases  of  various  organs.  It  is  a  very  frequent  phenomenon 
and  in  the  majority  of  cases  has  no  special  diagnostic  significance.  Some- 
times, however,  it  is  so  conspicuous  by  its  intensity  and  character  that  its 
diagnostic  value  is  enormous. 

Diseases  and  Conditions  that  Cause  Headache 

1.  Circulatory  Disorders.  Anaemia,  Hyperaemia. — In  all  forms  of 
anaemia  headache  is  a  common  occurrence.  It  is  particularly  observed 
when  an  effort,  is  made  by  the  patient.  In  aortic  diseases  cerebral  anemia 
occurs.     The  headache  is  then  accompanied  by  vertigo. 

In  passive  hyperaernia  pressure  on  the  neck  (tumors,  tight  collars,  etc.), 
in  active  hyperaernia  certain  drugs  (caffeine,  nitroglycerine,  alcohol), 
undue  physical  and  mental  effort,  violent  emotions,  overheated  room, 
cardiac  diseases,  especially  mitral  lesions,  are  the  causes  of  pain  in  the  head. 

2.  Meningitis  (localized  or  diffuse)  and  meningeal  hemorrhages,  also 
acute  encephalitis. 

3.  Toxaemia  in  infectious  diseases.  Perverted  metabolism,  such  as 
met  with  in  diabetes,  gout,  chronic  rheumatism,  nephritis,  albuminuria, 
uraemia,  gastro-intestinal  disorders  (constipation);  intoxications,  such  as 
lead,  alcohol,  tobacco,  opium,  carbonic  acid  gas. 

4.  Syphilis  through  a  degenerative  condition  of  the  blood  vessels  or 
gummatous  deposits  in  the  meninges. 

5.  Increased  Intracranial  Pressure  through  tumors,  aneurisms  of 
the  brain  (see  Tumors  of  Brain). 

6.  Hysteria,  Neurasthenia. 

7.  Traumatism  of  the  head. 

8.  Diseases  of  the  skull  and  of  its  cavities  (nasal,  frontal,  etc.). 

9.  Reflex  irritation,  such  as  errors  of  refraction,  naso-pharyngeal 
adenoids,  diseases  of  genitalia,  etc. 

Pathogenesis. — It  is  generally  conceded  that  headache  is  directly  due 
to  an  irritation  of  the  sensory  filaments  distributed  in  the  meninges  of 


536  HEADACHE 

the  brain,  especially  the  dura.  In  organic  cases  the  irritation  is  caused  by 
some  intracranial  disease.  In  the  functional  cases  some  irritant  (toxic 
or  other)  circulating  in  the  blood  vessels  reaches  the  sensory  ends  of  the 
meningeal  nerve-supply. 

Character  of  Headache  in  Various  Diseases 

1 .  Headache  due  to  anaemia,  chlorosis  or  loss  of  blood  is  usually  diffuse 
and  accompanied  by  a  sensation  of  pressure.  It  is  severe  and  aggravated 
upon  an  effort.     Vertigo  is  frequently  present. 

In  hyperemia  (passive  or  active)  the  headache  is  diffuse,  continuous 
and  of  a  throbbing  nature.  Horizontal  position  aggravates  it.  In  cerebral 
congestion  caused  by  a  cardiac  lesion,  there  is  a  venous  stasis  and  aedema 
of  the  brain.  The  headache  is  accompanied  by  somnolence  and 
even  delirium. 

2.  In  Meningitis  the  headache  may  be  localized  or  generalized.  It 
is  usually  continuous  and  presents  exacerbations.  In  this  disease  the 
headache  is  of  diagnostic  importance,  especially  in  the  epidemic  cere- 
brospinal or  tubercular  form  of  meningitis,  in  which  it  is  a  very  early 
symptom  and  of  great  severity. 

In  tubercular  meningitis  headache  is  less  intense  than  in  the  epidemic 
meningitis.  In  the  latter  the  pain  extends  down  to  the  neck  and  spine. 
In  both  forms  the  headache  is  persistent. 

In  Pachymeningitis  the  pain  in  the  head  is  at  first  localized. 

3.  In  infectious  diseases  headache  appears  in  the  prodromal  period 
and  usually  persists  through  the  entire  course  of  the  disease.  It  is  usually 
dull  and  continuous. 

In  influenza  the  headache  is  diffuse  and  particularly  marked  in  the 
frontal  sinuses.  This  disease  is  frequently  followed  by  headache  of 
unusual  severity. 

Headache  caused  by  poisons  is  usually  frontal,  dull  and  quite  severe. 
It  persists  even  after  the  other  symptoms  have  disappeared. 

4.  Imperfect  metabolism  of  gastro-intestinal  origin  (constipation) 
produces  a  temporal  or  occipital  headache. 

5.  Syphilitic  headache  is  mostly  nocturnal  in  character.  It  is  dull, 
continuous,  with  nocturnal  exacerbations.  It  may  be  general  or  confined 
to  one  side  of  the  skull.  It  is  frontal  or  parietal  most  frequently.  In  the 
latter  case  the  skull  is  tender  on  percussion.  Headache  is  the  earliest 
symptom  of  cerebral  syphilis.  It  is  due  to  involvement  of  the  meninges 
by  gummata.  It  is  usually  relieved  by  antisyphilitic  remedies,  but  recurs 
readily. 


HEADACHE  537 

6.  Headache  is  a  very  significant  symptom  in  intracranial  diseases, 
as  tumor,  abscess,  etc.  It  is  of  unusual  severity  and  persistent.  Percus- 
sion or  deep  pressure  on  the  skull  provokes  pain.  In  case  of  abscess  due 
to  an  ear  lesion,  a  disease  of  the  nose,  of  frontal  sinus,  etc.,  the  pain  will 
be  localized  in  the  corresponding  regions. 

In  tumors  of  the  cerebellum  the  headache  is  dull,  boring  and  sometimes 
sharp,  but  always  intense.  It  is  localized  frequently  in  the  occipital 
region. 

7.  In  Neurasthenia  the  headache  is  in  the  form  of  continuous  pressure 
or  constriction;  it  resembles  a  sensation  of  a  tight  iron  band  around  the 
head.  It  is  usually  diffuse,  but  it  may  be  also  localized.  Neurasthenics 
often  complain  of  various  distressing  sensations  in  the  head,  as  heat, 
emptiness  or  else  fullness,  which  alternate  with  or  accompany  headache. 

In  Hysteria  the  headache  is  in  the  vertex.  It  is  boring  and  localized, 
as  "clavus,"  viz.    a  sensation  of  a  nail  being  driven  into  the  head. 

8.  In  traumata  of  the  head  and  diseases  of  the  skull  the  pain  is  usually 
circumscribed  and  corresponds  to  the  injured  or  diseased  area,  although 
it  may  spread  from  the  latter  and  become  diffuse. 

9.  Reflex  Headache  is  quite  common.  When  it  is  due  to  errors  of 
refraction,  it  is  frontal  or  temporal.  Nasal  diseases  give  headache  in  the 
vertex  or  temporal  region.  Persistent  headache  is  present  in  diseases  of 
the  auditory  apparatus.  A  thorough  examination  in  each  case  of  head- 
ache for  possible  reflex  causes  is  necessary. 

Treatment* — Determination  of  the  cause  of  headache  is  the  first  indi- 
cation before  the  treatment  is  instituted.  The  above  described  etiological 
factors  should  be  borne  in  mind.  Every  case  must  be  scrupulously  ex- 
amined, as  the  complete  removal  of  the  headache  presents  frequently 
great  difficulties.  As  soon  as  the  cause  of  headache  is  ascertained,  all 
medical  or  surgical  measures  should  be  employed  for  its  prompt  removal. 

In  cases  with  circulatory  disturbances  the  latter  must  be  relieved. 
In  anaemia  rest,  absolute  or  partial,  according  to  the  case,  is  necessary. 
Good  nutritious  food,  iron  and  arsenic  are  appropriate.  In  hyperaemia, 
besides  the  removal  of  the  original  cause,  purgatives  are  indicated.  In 
meningitis  purgations  and  local  abstraction  of  blood  are  useful. 

In  gastro-intestinal  disorders  their  immediate  correction  is  necessary. 
Regulation  of  diet,  avoidance  of  nitrogenous  food,  of  intoxicants,  light  but 
nutritious  food  according  to  the  case,  daily  evacuations,  also  administra- 
tion of  hydrochloric  acid  in  case  of  diminished  secretion  in  the  stomach, 
and  of  an  alkaline  in  case  of  increased  secretion  of  hydrochloric  acid,  ad- 
ministration of  a  stimulant  stomachic  in  case  of  sluggish  digestion — all 
these  means  are  indicated  for  treatment  of  headache.     Headache  caused 


538  HEADACHE 

by  certain  poisons  will  be  treated  by  measures  of  elimination  and  anti- 
dotes, also  by  removal  of  the  patient  from  exposure  to  the  poisons.  In 
cases  of  lead  poisoning  besides  systematic  elimination,  iodides  are  beneficial. 

Symptomatic  Treatment  of  Headache. — Relief  of  pain  can  be  ob- 
tained from  coal-tar  products.  Aspirin,  sodium  salicylate,  antipyrin, 
bromides,  are  useful.  A  combination  of  aspirin,  gr.  v,  phenacetine,  gr.  ii, 
and  caffein  citrate,  gr.  j,  administered  every  two  hours,  has  given  me  very 
satisfactory  results.  In  case  of  malaria  quinine  is  advisable.  For  head- 
ache of  syphilitic  nature  salvarsan,  mercurials  or  iodides  or  both  will  give 
relief. 

Local  application  of  extreme  heat  or  cold,  counter-irritation,  appli- 
cation of  menthol,  alcohol,  chloroform,  ether,  may  give  some  relief. 

Complete  isolation  with  absolute  rest  sometimes  gives  relief. 

In  organic  headache  (tumor,  abscess,  etc.)  surgical  measures  are  the 
only  means,  as  it  is  unusually  rebellious  to  internal  medications. 

Morphia  should  be  avoided,  as  the  pernicious  habit  is  easily  acquired. 
In  infectious  diseases  headache  is  caused  by  a  slight  inflammation  of  the 
meninges,  which  is  indicated  by  increase  of  pressure  and  of  albumen  in 
the  cerebro-spinal  fluid.  In  such  cases  a  lumbar  puncture,  and  with- 
drawal of  10-20-30  c.c.  of  cerebro-spinal  fluid,  may  give  considerable 
relief.  Recent  observations  of  Roger  and  Baumel  (Revue1,  de  Med.  No. 
1,  1 91 3)  are  very  conclusive. 

Muscular  or  Indurative  Headache 

This  form  of  headache  is  due  to  a  rheumatic  infiltration,  a  chronic 
myositis  of  the  muscles  of  the  neck.  The  cervical  muscles  are  swollen 
and  tender  to  touch.  The  tenderness  exists  at  the  insertions  of  the  ten- 
dons, spinous  processes,  mastoid  processes,  clavicle  and  the  muscles 
themselves.  In  subacute  myositis,  the  swelling  of  the  muscles  has  an 
elastic  consistency.  In  chronic  myositis  hard  nodules  are  felt  in  the 
muscles.  The  skin  covering  the  swollen  muscles  is  infiltrated  and  thick. 
Histological  studies  show  that  the  infiltrations  are  due  to  a  proliferation 
of  the  connective  tissue,  at  first  cedematous,  later  fibrous  and  accompanied 
by  endoperiarteritis  (Lorenz  and  Stockman).  It  is  therefore  a  trophic 
disturbance. 

The  above  mentioned  tenderness  is  present  not  only  in  the  insertions 
of  the  tendons  but  also  in  the  infiltrated  masses.  Muller  (Deut.  Ztschr.  f. 
Nerv.,  19 10)  calls  attention  to  hypertonicity  of  the  affected  muscles:  the 
muscles  of  the  neck  are  rigid  and  when  the  patient  wishes  to  turn  his  head, 
the  entire  body  moves  with  it.     The  pain  produced  by  muscular  induration 


MIGRAINE 


539 


may  be  diffuse  and  affect  the  entire  head,  or  migrainic  affecting  one  side 
of  the  head  or  else  neuralgic.  These  three  varieties  may  alternate  in  the 
same  individual. 

Pathogenesis. — The  headache  can  be  explained  either  by  an  involve- 
ment of  the  sensory  fibers  passing  through  the  infiltrated  muscles,  or  by 
irritation  of  the  sympathetic.  In  fact  Norstrom  and  Hartenberg  observed 
in  migrainic  patients  a  swelling  of  the  upper  and  middle  cervical  sympa- 
thetic. It  stands  to  reason  that  in  swelling  of  the  muscles  of  the  neck  the 
sympathetic  is  being  irritated,  hence  the  headache.  Miiller  also  believes 
that  the  swollen  muscles  compress  the  jugular  veins,  the  return  circulation 
is  interfered  with,  a  cerebral  congestion  is  the  result,  hence  the  headache. 

Hartenberg  believes  that  the  chief  cause  of  these  muscular  infiltrations 
lies  in  an  insufficiency  of  arterial,  venous  and  lymphatic  circulation  which 
is  the  result  of  an  insufficiency  of  muscular  activity.  Cold,  which  decreases 
the  nutritive  and  circulatory  conditions,  is  capable  to  produce  the  lesion. 

Treatment. — Avoidance  of  cold,  general  hygiene,  hydrotherapy,  are 
measures  not  to  be  neglected.  Galvanism  applied  to  the  affected  muscle 
in  the  beginning  is  very  useful.  Massage  should  be  employed  only  after 
the  acute  symptoms  have  subsided;  it  is  contraindicated  when  the  muscles 
are  very  tender.  Internally  cannabis  indica  has  been  advised  by  Harten- 
berg in  very  rebellious  cases. 

Migraine.    Hemicrania 

Under  this  name  is  known  a  variety  of  headache  which  occurs  in 
paroxysms,  is  confined  to  one  side  of  the  head,  and  is  accompanied  by 
nausea,  vomiting,  vertigo  and  ocular  phenomena. 

Symptoms. — The  onset  is  usually,  but  not  always,  preceded  by 
prodromal  symptoms.  They  may  be  either  depression,  apathy,  somno- 
lence or,  on  the  contrary,  exaltation  with  a  ravenous  appetite. 

The  pain  sets  in  ordinarily  in  the  morning,  when  the  patient  gets 
.awake.  At  first  it  is  dull,  but  gradually  increases  as  the  day  advances. 
It  soon  becomes  severe  and  even  intolerable.  The  patient  compares  it 
to  a  tearing,  breaking,  boring.  The  least  motion  of  the  head  or  the  act 
of  coughing,  sneezing,  exaggerates  it.  It  is  confined  mostly  to  one  side 
of  the  head  and  to  the  left  most  frequently.  It  may  also  spread  to  the 
other  side.  It  may  also  alternate  in  various  attacks.  It  may  appear 
first  in  the  orbital  or  temporal  region  and  from  this  point  spread  to  the 
entire  half  of  the  head. 

During  the  attack  an  irritation  of  any  of  the  special  senses  aggravates 
the  pain.     A  loud  sound,  a  sharp  odor,  a  bright  light,  become  intolerable. 


540 


MIGRAINE 


For  this  reason  the  patient  instinctively  isolates  himself,  closes  up  the 
windows,  the  doors,  and  seeks  quietness.  When  the  headache  reaches  the 
maximum,  vomiting  ocurs.  This  is  a  frequent  symptom  and  it  may  take 
place  also  at  the  onset  of  an  attack.  It  is  accompanied  by  vertigo.  The 
patient  rejects  a  bilious,  mucous  fluid.  It  is  brought  on  by  the  least 
amount  of  fluid  or  food  and  sometimes  it  occurs  before  breakfast.  There 
may  be  several  attacks  of  vomiting.  Sometimes  an  attack  of  vomiting 
ends  the  attack  of  migraine.  The  patient  then  feels  considerably  relieved 
and  the  headache  rapidly  disappears.  In  a  large  number  of  cases  the 
final  vomiting  spell  is  followed  by  a  deep  sleep.  When  the  patient  gets 
awake,  he  is  totally  relieved  and  feels  well.  Sometimes,  however,  there  is 
a  feeling  of  lassitude  and  a  dullness  in  the  head  which  persist  for  several 
days. 

As  accessory  symptoms  of  migrainic  attacks  can  be  mentioned  lachry- 
mation,  unilateral  or  bilateral,  sweating,  polyuria,  coldness  of  the  extrem- 
ities and  exceptionally  hemorrhages. 

Forms  of  Migraine 

i.  Ophthalmic  Migraine. — It  is  characterized  by  visual  disturbances 
at  the  onset  of  an  attack.  The  most  important  among  them  is  a  scotoma 
(scotoma  scintillans) .  It  is  a  dark-grayish  spot  in  the  visual  field,  sur- 
rounded by  a  bright  border  of  various  colors.  Sometimes  instead  of  a 
scotoma  the  visual  field  is  covered  by  glaring  zig-zag  lights.  In  other 
cases  there  is  a  distinct  blindness  of  the  visual  field  in  the  form  of  homon- 
ymous hemianopsia.  There  may  be  also  a  transitory  amaurosis,  photo- 
phobia with  interocular  pain.  In  some  cases  the  migraine  may  be  accom- 
panied or  followed  by  temporary  ophthalmoplegic  symptoms  such  as 
strabismus,  diplopia,  ptosis,  loss  of  accommodation.  In  exceptional  cases 
they  remain  permanent. 

Ophthalmic  migraine  may  be  associated  with  motor,  sensory  and 
psychic  phenomena. 

A  paresis  of  the  side  opposite  to  the  seat  of  head-pain,  paraesthesias 
on  one  or  both  sides  of  the  body  are  sometimes  observed. 

Aphasia  (motor)  with  agraphia  is  occasionally  observed.  One  of  my 
patients  had  three  attacks  of  a  mild  right  hemiplegia  with  slight  aphasia. 
During  these  attacks  the  right  knee-jerk  was  increased  and  the  paradoxical 
reflex  was  evident.     These  manifestations  lasted  but  a  few  hours. 

Cerebellar  ataxia  with  its  characteristic  disturbance  of  equilibration 
was  observed  by  Oppenheim. 

All  these  phenomena  are  usually  transitory  and  last  a  short  lime; 


MIGRAINE  .  541 

they  may  precede  the  headache  and  vomiting  or  persist  during  the  entire 
attack  of  migraine. 

The  psychic  symptoms  are  various.  There  may  be  only  a  temporary 
amnesia,  visual  hallucinations,  depression,  apathy,  which  disappear  with 
the  attack.  There  may  also  develop  true  psychoses,  as  Krafft-Ebing, 
Mingazzini  and  myself  have  shown. 

In  my  recent  study  {Journal  of  American  Medical  Association,  January 
5,  1907)  of  the  subject,  I  described  twelve  personal  cases  of  typical  psy- 
choses observed  in  migrainic  attacks.  In  all  of  them  I  have  invariably 
found  three  mental  states,  viz.  (1)  Confusion,  (2)  stupor,  with  hallucina- 
tions and  unsystematized  delusions,  and  (3)  delirium.  The  hallucinations 
were  mostly  visual,  although  auditory  and  gustatory  were  also  found  in 
some  of  the  twelve  cases.  The  confusional  state  predominated  in  all  my 
patients.  It  was  quite  frequently  accompanied  by  illusions  of  identity, 
incoherence  of  thoughts  and  disturbance  of  orientation.  The  delusions 
were  of  a  fleeting  character.  In  the  majority  of  cases  the  mental  symptoms 
developed  during  the  attacks  when  the  headache  reached  its  climax  and 
disappeared  with  the  headache. 

2.  Hemicrania  Sympathico-tonica  (White  Migraine) . — It  is  char- 
acterized by  symptoms  of  irritation  of  the  cervical  sympathetic  nerve,  viz. 
pallor  of  the  integument  of  the  head  on  the  side  of  the  pain;  the  temporal 
artery  is  of  high  tension,  the  local  temperature  is  lowered;  there  is  also 
retraction  of  the  eyeglobe  and  mydriasis. 

3.  Hemicrania  Sympathico-paralytica  (Red  Migraine)  is  characterized 
by  symptoms  of  paralysis  of  the  cervical  sympathetic  nerve,  viz.  redness 
of  the  integument  of  the  head  on  the  affected  side,  lachrymation,  photo- 
phobia, contraction  of  the  pupil,  elevation  of  the  local  temperature,  uni- 
lateral hyperhidrosis;  the  temporal  artery  is  distended. 

4.  Migraine  in  its  Relation  to  Epilepsy. — It  is  well  known  that  both 
neuroses  may  be  associated  and  that  the  first  may  be  equivalent  to 
epileptic  seizures.  That  the  analogy  between  them  is  considerable  can 
be  seen  from  this  fact  that  in  both  the  attacks  are  sometimes  preceded  by 
auras  (motor,  sensory,  psychic),  that  the  onset  may  be  sudden,  that  an 
attack  is  followed  by  a  sense  of  exhaustion  and  sometimes  by  unilateral 
paralysis  or  paresis.  Finally  the  abortive  cases  of  migraine  (see  below) 
are  almost  identical  to  attacks  of  petit  mal. 

5.  Abortive  Migraine. — It  consists  of  incomplete  attacks  (without 
vomiting  or  vertigo)  or  else  sudden  attacks  of  vomiting  without  headache. 
Sometimes  it  is  only  an  attack  of  vertigo.  This  form  alternates  frequently 
with  the  typical  attacks  of  migraine. 

Course,  Duration,  Prognosis.— A  typical  attack  commences  in  the 


542  MIGRAINE 

morning,  gradually  increases  in  severity  and  terminates  in  the  evening 
with  vomiting  and  sleep.  When  he  wakes  up,  he  feels  refreshed  and  well. 
An  individual  attack  may  last  from  a  couple  of  hours  to  twenty-four  or 
forty-eight  hours.  In  some  patients  there  is  a  great  regularity  in  the 
periodicity  of  attacks.  In  such  cases  it  comes  on  once  every  month  or 
every  two  months.  In  women  it  may  coincide  with  menstruation.  In 
other  cases  the  attacks  are  irregular  and  develop  only  after  some  excess 
(alcoholic  or  other)  or  after  some  indulgence  in  eating.  The  disease  lasts 
many  years  or  even  the  entire  life.  Usually  with  advent  of  old  age  it 
decreases  in  intensity  and  disappears.  Sometimes  an  intercurrent  disease, 
a  trauma  or  shock,  makes  migraine  disappear  completely  or  for  many  years. 
In  such  cases  some  other  neurosis  (hysteria,  epilepsy,  etc.)  develops  instead 
of  it.  In  some  patients  after  many  years  of  periodical  and  regular  attacks 
the  interval  between  them  becomes  shorter  and  shorter  and  finally  the 
headache  becomes  continuous.  A  sort  of  migrainic  status  is  thus  estab- 
lished.    The  Prognosis  is  unfavorable  as  to  recovery,  but  not  to  life. 

Diagnosis. — The  symptoms  ordinarily  are  typical  enough  for  a  correct 
diagnosis. 

In  cerebral  tumors  there  are  also  severe  headache  and  vomiting,  but 
there  is  no  interval  of  "well  being"  characteristic  of  migraine.  Besides, 
the  changes  in  the  eyegrounds  will  aid  in  the  diagnosis.  Tabes  and  Paresis 
may  present  migraine  as  one-  of  their  initial  symptoms.  Syphilitic  and 
malarial  migraine  will  be  promptly  relieved  by  mercurials  and  quinine, 
respectively.  The  diagnosis  between  ophthalmic  migraine  and  neuralgia 
of  the  ophthalmic  branch  of  the  trigeminus  is  not  always  easy.  The  fol- 
lowing differential  signs  may  be  useful.  In  migraine  there  is  nausea,  vom- 
iting. In  neuralgia  they  are  absent.  In  migraine  the  pain  is  continuous 
for  several  hours.  In  neuralgia  it  is  in  paroxysms.  In  migraine  remissions 
are  of  long  duration — hours  or  days.  In  neuralgia  they  are  very  brief. 
In  migraine  photophobia,  but  not  in  neuralgia.  In  migraine  scotomata, 
but  not  in  neuralgia.  In  migraine  the  pain  often  spreads  to  the  other 
side  of  the  head.     In  neuralgia  the  pain  is  strictly  confined  to  one  side. 

Etiology  and  Pathogenesis. — The  most  important  element  in  the 
etiology  is  predisposition.  The  disease  is  very  frequently  found  to  be 
hereditary.  A  neuropathic  personal  or  family  history  is  frequently 
found.  An  association  of  migraine  with  an  arthritic  diathesis  is  a  common 
observation. 

The  most  recent  researches  in  the  domain  of  physiologic  chemistry 
lead  to  the  view  that  migraine  finds  its  explanation  in  autointoxication. 
Whether  it  is  uric  acid  or  a  special  ferment  or  ptomain  is  not  completely 
elucidated.     The  fact  is  that  in  a  large  majority  of  cases  there  is  an  ele- 


MIGRAINE  543 

ment  of  gastro-intestinal  disorder  and  that  arthritic,  gouty,  asthmatic, 
obese  and  constipated  individuals  are  most  frequently  affected. 

Our  recent  studies  on  the  function  of  the  ductless  glands  permit  also 
to  suppose  that  their  derangement  is  apt  to  play  a  certain  part  in  the 
causation  of  migraine. 

Suffice  it  to  mention  the  fact  that  in  pregnancy,  for  example,  there 
is  a  functional  hyperactivity  of  the  thyroid  gland  and  migrainic  patients 
are  frequently  free  from  attacks  of  migraine  at  that  time.  General  lassi- 
tude, anorexia,  constipation,  obesity,  falling  of  hair,  are  observed  in 
migrainic  individuals,  also  in  cases  with  diminished  thyroid  function. 

A  poison,  whatever  its  source  may  be,  circulating  in  the  blood  vessels 
irritates  the  sensory  filaments  of  the  dura  and  produces  the  syndrome  of 
migraine. 

As  exciting  causes  may  be  mentioned:  disturbances  of  digestion, 
excesses,  masturbation,  irregular  sleep,  physical  and  mental  fatigue, 
emotions,  lack  of  fresh  air,  certain  odors,  loud  sounds.  Pregnancy  may 
provoke  or  arrest  the  attacks.     Women  are  more  often  affected  than  men. 

Treatment. — A  patient  suffering  from  migraine  should  be  energetically 
treated  between  the  attacks.  Proper  measures  taken  at  that  time  will 
in  a  great  many  cases  succeed  in  increasing  the  intervals  between  individual 
attacks  and  in  some  cases  accomplish  a  complete  cure. 

As  said  above,  migraine  is  due  in  the  majority  cf  cases  to  autointoxi- 
cation. It  is  therefore  toward  the  latter  that  our  therapeutic  efforts 
must  be  directed.  Each  migrainic  individual  should  first  of  all  be  put 
on  a  special  diet  and  follow  a  certain  mode  of  living.  It  has  been  my 
practice  to  remove  from  the  diet  meats,  stimulants  of  any  kind,  including 
tea  and  coffee,  sweets,  pastry.  Starchy  food  is  allowed  only  in  extremely 
small  quantities.  Milk  is  of  course  desirable,  but  it  is  not  tolerated 
by  every  patient;  in  the  latter  case  I  substitute  it  by  either  butter  milk, 
skimmed  milk,  kephir,  koumyss  or  else  by  plain  water.  In  a  great  many 
cases,  however,  I  succeeded  in  having  the  patient  accustomed  to  milk  by 
giving  it  at  first  in  very  small  quantities  and  then  very  gradually  increas- 
ing the  amount.  The  amount  of  food  of  each  meal  should  be  moderate 
and  taken  regularly.  Fruit,  eggs,  milk,  green  vegetables,  crackers,  cus- 
tards, junkets,  gelatin,  are  the  only  articles  allowed.  As  drink,  plain  water 
or  some  gaseous  mineral  water,  like  apollinaris,  lemonade,  are  permissible. 
Saline  purgatives  should  be  administered  at  first  twice  a  week  and  later 
once  a  week  for  a  long  period. 

Hydrotherapy  in  the  form  of  cold  shower  baths  of  a  minute's  dura- 
tion and  followed  by  massage  twice  a  day  is  very  beneficial. 

Daily  walks  two  or  three  times  a  day  of  an  hour  each  are  advisable. 


544        '  VERTIGO 

A  quiet  life,  free  from  undue  emotions,  retiring  early,  are  also  necessary. 

Tobacco  must  be  used  very  moderately  and,  if  possible,  abandoned. 

As  to  medications,  a  stomachic,  as  nux  vomica  together  with  hydro- 
chloric acid  and  gentian,  taken  regularly  before  meals  is  of  benefit.  Any 
dyspeptic  disturbance  should  be  corrected  and  treated  accordingly.  A 
thorough  examination  of  all  the  organs  must  be  made  as  early  as  possible 
and  treated  if  necessary.  In  case  of  anaemia,  iron  and  arsenic  should  be 
given.  Arsenic  is  highly  praised  by  Oppenheim.  The  urine  should  be 
frequently  examined.  Any  diseased  condition  of  nasal,  pharyngeal  and 
other  cavities,  errors  of  refraction,  diseases  of  the  ears,  etc.,  must  be  taken 
care  of. 

When  an  attack  of  migraine  occurs,  all.  food  must  be  immediately 
withdrawn,  the  patient  put  to  bed,  the  room  made  dark  and  all  noises 
avoided.  An  ice  cap  or  else  a  hot  water  bag  are  to  be  applied  to  the  head. 
Application  of  menthol,  a  spray  of  ether  or  alcohol  to  the  forehead  or 
temples  may  give  some  relief.  The  vomiting  is  sometimes  controlled  by 
internal  administration  of  small  pieces  of  ice  or  chloroform  water. 

All  these  means  should  be  tried  before  medications  in  every  case  of 
migraine.  The  following  are  the  remedies  advised  for  combating  the  pains : 
Coal-tar  products,  aspirin,  salicylates,  phenacetin,  antipyrin.  A  combina- 
tion which  has  given  me  satisfactory  results  in  many  cases  is:  Aspirin, 
gr.  v,  phenacetin,  gr.  ii,  caffeine  citrate,  gr.  j,  to  be  taken  every  hour  or 
two  hours  until  relieved.  Aspirin  and  codein  form  a  good  combination. 
Bromides  succeed  sometimes  when  other  drugs  fail. 

In  the  sympathico-tonic  form  of  migraine  (see  above)  nitro-glycerine 
is  useful.  In  the  sympathico-paralytic  form  (see  above)  ergot  is  advisable. 
Amyl-nitrite  in  inhalations  (five  drops  on  a  handkerchief)  sometimes  cuts 
short  an  attack.    Morphia  should  be  avoided. 

In  the  discussion  on  the  pathogenesis  of  migraine  its  relation  to  dis- 
turbances of  thyroid  function  was  indicated.  In  a  series  of  cases  studied 
and  reported  by  me  in  the  Therapeutic  Gazette,  1907,  I  showed  the  very 
satisfactory  results  obtained  in  the  treatment  of  migraine  by  thyroid 
extract,  when  every  other  medication  failed.  This  treatment  will  yield 
good  results  only  in,  so  to  speak,  "thyroid  cases,"  viz.  in  cases  with  a 
deficient  thyroid  function. 

VERTIGO 

Vertigo  is  not  a  disease  but  a  symptom.  It  may  be  encountered  in 
various  diseases  and  conditions.  In  Meniere's  disease  it  is  the  most  con- 
spicuous symptom. 


VERTIGO 


545 


Nature  of  Vertigo. — The  relation  of  the  body  to  surrounding  objects 
determines  the  so-called  "sense  of  space."  Should  this  relation  be  dis- 
turbed, our  orientation  and  equilibration  in  the  space  will  become  irregular 
and  vertigo  will  ensue. 

In  vertigo  the  orientation  is  first  lost  and  the  loss  of  equilibrium 
follows. 

Orientation  is  the  result  of  centripetal  function.  The  pathway  con- 
trolling it  is  composed:  (i)  of  sensory  fibers  going  from  the  periphery 
through  the  spinal  ganglia  and  posterior  columns,  also  direct  cerebellar 
tract  of  the  cord  to  their  termination  in  the  cortex  of  the  cerebrum  and 
cerebellum,  respectively;  (2)  of  auditory  fibers  originating  in  the  labyrinth 
and  ending  either  in  the  temporal  lobe  of  the  brain  (cochlear  nerve)  or 
in  the  nucleus  of  Deiter  in  the  medulla  (vestibular  nerve). 

Equilibration  is  a  centrifugal  function.  Its  pathway  consists  of  fibers 
of  the  pyramidal  tract  and  of  the  descending  cerebellar  tract,  also  of 
Monakow's  bundle,  which  all  end  in  the  anterior  cornua  of  the  spinal  cord. 

Disturbance  in  the  harmonious  function  of  those  pathways  or  centers 
of  orientation  and  equilibration  results  in  vertigo. 

Diseases  and  Conditions  in  which  Vertigo  Occurs 

1.  Disorders  in  the  Hearing  Apparatus. — Any  disease  of  the  ear,  from 
a  grave  affection  of  the  labyrinth  to  a  simple  accumulation  of  cerumen  in 
the  external  ear,  may  produce  Vertigo.  The  most  important  form  of  aural 
vertigo  is  the  so-called  Meniere's  disease.  Suddenly  the  patient  hears  a 
noise  in  the  ear,  which  is  immediately  followed  by  dimness  of  vision  or 
double  vision  and  sometimes  nystagmus.  Vertigo  sets  in  at  once  and  the 
patient  falls.  At  that  time  he  feels  the  floor  sinking  and  himself  revolving 
in  a  circle.  He  is  nauseated  and  begins  to  vomit.  Headache,  coldness  of 
the  skin,  pallor,  accompany  the  vertigo.  After  the  attack  is  over,  the 
noise  in  the  ear  persists.  If  the  attacks  are  frequent,  hypoacusia  or 
deafness  gradually  develops  and  vertigo  becomes  chronic. 

The  paroxysms  usually  last  a  few  minutes.  They  may  occur  every 
day,  week,  month  or  even  at  longer  intervals.  The  disease  may  last  an 
indefinite  time,  but  when  recovery  takes  place,  total  deafness  is  established. 

The  disease  is  probably  due  to  a  labyrinthine  involvement  (progressive 
degeneration  of  the  nerve-ends) ;  a  lesion  of  the  cochlea  causes  deafness, 
and  of  the  semicircular  canal  vertigo.  The  labyrinth  is  innervated  by  the 
vestibular  branch  of  the  eighth  nerve.  This  branch  is  connected  with  the 
cerebellum,  center  of  equilibrium.  The  ocular  symptoms  are  due  to  the 
anatomical  relation  between  the  vestibular  nerve  and  the  nucleus  of  the 

35      . 


546  VERTIGO 

oculomotor  nerve.     To  sum  up,  vertigo,  tinnitus  auris,  hypoacusia,  or 

deafness  constitute  the  chief  characteristic  symptoms  of  Meniere's 
disease.  Nausea,  vomiting  and  nystagmus  are  not  necessarily  always 
present. 

Diseases  of  the  inner  ear,  of  the  middle  ear,  of  the  external  ear,  of  the 
acoustic  nerve,  injuries  of  the  petrous  bone,  meningitis,  tabes,  syphilis, 
gout,  anaemia,  arteriosclerosis,  may  be  accompanied  by  Meniere's  symp- 
tom-group, but  it  has  also  been  observed  in  persons  free  from  any  disease. 
In  such  cases  a  vaso-motor  disturbance  in  the  labyrinth  is  probably  at 
fault. 

Forms  of  Meniere's  Disease,  (a)  Charcot  described  a  continuous 
form  of  the  disease.  The  auditory  disorder  and  the  hypoacusia,  also  the 
vertiginous  state  are  permanent,  so  that  the  patient's  gait  is  that  of  cere- 
bellar type  (gait  of  an  inebriate).  In  addition  to  this  continuous  condi- 
tion the  patient  has  paroxysms  of  the  acute  form. 

(b)  In  the  mild  form  of  Meniere's  disease,  which  is  the  most  frequent, 
all  the  typical  symptoms  are  present  but  they  are  exceedingly  slight 
Here  the  manifestations  are  not  always  referred  to  the  real  cause.  Stom- 
ach, kidney,  uterus,  anaemia  are  frequently  incriminated. 

(c)  Incomplete  forms  (formes  frustes)  were  described  by  Frankl- 
Hochwart.  One  or  two  chief  elements  of  the  disease  "may  be  wanting. 
Thus  we  observe:  a  form  with  hypoacusia,  a  form  without  tinnitus,  a  form 
with  a  mild  vertigo. 

(d)  Apoplectic  form  is  probably  due  to  a  labyrinthine  hemorrhage. 
Its  onset  is  sudden.  It  is  usually  accompanied  by  a  complete  and  irre- 
mediable loss  of  hearing.  This  is  the  form  described  originally  by  Meniere. 
Loss  of  consciousness  is  sometimes  present.  It  is  usually  met  with  in 
individuals  suffering  from  syphilis,  tabes,  arteriosclerosis,  nephritis, 
infectious  diseases.  In  traumatism  of  the  ear  labyrinthine  hemorrhage 
and  therefore  apoplectic  form  of  Meniere's  disease  may  occur. 

In  making  a  diagnosis  of  Meniere's  disease,  syncope,  hysterical  or 
epileptic  attacks,  indigestion,  should  be  thought  of.  To  determine  laby- 
rinthine involvement,  the  tests  described  in  the  chapter  on  Nystagmus 
must  be  taken  into  consideration  (seepage  363.) 

2.  Disorders  in  the  Visual  Apparatus. — Ocular  palsies,  diplopia, 
nystagmus,  sudden  passage  from  obscurity  to  light,  are  common 
causes  of  vertigo,  and  if  the  latter  is  severe,  nausea  and  vomiting  may 
follow. 

3.  Disturbances  in  the  Central  Nervous  System. — Tabes,  multiple 
sclerosis,  may  be  accompanied  by  vertigo.  In  the  latter  affection  it  is 
quite  frequent  and  it  is  an  indication  of  bulbar  involvement. 


VERTIGO  547 

In  diseases  of  the  brain  (tumors)  and  especially  of  the  cerebellum 
vertigo  is  one  of  the  most  important  diagnostic  symptoms.  In  cerebellar 
conditions  the  vertigo  is  of  a  rotatory  type.  Softening  of  the  brain, 
circulatory  changes,  atheromatous  condition  of  the  cerebral  blood  vessels, 
are  accompanied  by  vertigo.  Diseases  of  the  medulla,  cerebellar  peduncles 
and  vestibular  nerve,  all  partaking  in  the  control  of  equilibrium,  will 
cause  vertigo. 

4.  Disorders  of  Metabolism  (Toxic  Causes). — Nephritis,  gout,  dia- 
betes, gastro-intestinal  disturbances,  migraine,  are  not  infrequently  accom- 
panied by  vertigo. 

Infectious  diseases,  intoxications  (alcohol,  lead,  coffee,  tobacco, 
quinine),  intestinal  parasites  produce  vertigo. 

5.  Hysteria  and  neurasthenia,  exophthalmic  goiter,  mental  diseases, 
may  be  accompanied  by  vertigo. 

6.  Visceral  Disturbances. — In  cardiac  diseases,  especially  diseases  of 
the  aorta,  vertigo  is  not  infrequently  present.  Dilatation  of  the  stomach 
may  cause  dizziness,  but  here  the  latter  is  probably  due  to  auto-intoxica- 
tion. Reflex  vertigo  may  originate  in  the  uterus,  bladder,  liver,  kidney, 
larynx,  nasal  cavity. 

7.  Sea-sickness,  traumatism,  insolation  are  accompanied  by  vertigo. 

8.  Paralytic  vertigo  (Gerlier),  which  has  been  observed  in  Switzerland, 
is  characterized  by  vertigo,  weakness  of  the  limbs  and  of  the  muscles  of 
the  neck,  ptosis.  It  occurs  in  paroxysms.  Nothing  is  known  of  the  nature 
of  this  endemic  affection. 

Treatment. — The  treatment  of  vertigo  is  closely  connected  with  the 
management  of  the  diseases  in  which  it  occurs.  An  effort  must  be  made 
first  of  all  to  remove  the  cause. 

In  Meniere's  disease  Charcot's  advice  has  given  me  the  best  results 
in  some  cases.  Quinine  hydrochlorate  given  in  small  doses,  but  frequently 
repeated,  with  milk  diet  and  avoidance  of  stimulants  yield  very  satis- 
factory results.  The  administration  of  quinine  is  based  on  this  fact,  that 
vertigo  subsides  with  increase  of  deafness.  Quinine  weakens  the  hearing 
and  it  may  therefore  be  useful.  At  first  it  will  increase  the  noises  of  the 
ears  and  even  the  vertigo,  but  if  persisted  in,  it  will  eventually  relieve  the 
vertigo.  Aspirin,  sodium  salicylate,  pilocarpine  in  hypodermic  injections 
(5-10  minims  of  2  per  cent,  solution  every  other  day)  may  be  useful. 
Purgatives,  hot  foot-baths  and  bleeding  may  be  beneficial.  Babinski 
obtained  some  relief  from  lumbar  punctures.  Krause,  in  1902,  and 
Frazier,  in  1909,  attempted  intracranial  division  of  the  auditory  nerve 
for  relief  of  intractable  tinnitus  and  aural  vertigo  respectively.  The 
results  have  been  encouraging. 


CHAPTER  XXVI 

TRAUMATIC  NEUROSES  AND  PSYCHOSES 

RELATION  OF  ACCIDENTS  TO  FUNCTIONAL  NERVOUS  DISEASES  AND 
PSYCHOSES;  MEDICOLEGAL  CONSIDERATIONS 

The  modern  requirements  in  every  sphere  of  human  activities  are 
highly  contributory  to  accidental  injuries.  Traumata  may  lead  to  sur- 
gical and  nervous  disturbances.  As  we  will  be  concerned  exclusively 
with  the  latter,  it  is  necessary  first  to  emphasize  what  factors  are  the  most 
frequent  causes  of  this  disorder. 

Among  all  accidents,  those  caused  by  conveyances  (railroads  or  trolley 
cars)  give  the  largest  contingent  of  victims.  Organic  injuries,  such  as 
fracture  or  dislocation  of  the  vertebrae,  tearing  of  the  spinal  cord  or  of 
peripheral  nerves,  fracture  of  the  skull,  followed  by  tearing  of  or  hemor- 
rhages in  the  cerebral  tissue  are  discussed  in  their  respective  chapters. 
Functional  nervous  disorders  and  psychoses  exclusively  will  be  discussed 
here.  The  subject  is  an  extremely  important  one  for  two  reasons:  (i) 
traumatic  functional  neuroses  are  exceedingly  frequent;  (2)  the  neuroses 
are  very  frequently  misunderstood,  and  therefore  a  proper  estimate  of  their 
value  is  not  always  given. 

The  functional  nervous  diseases  produced  or  perhaps  brought  out  by 
such  accidents  are:  hysteria,  neurasthenia,  chorea,  paralysis  agitans, 
amnesia.  A  review  of  the  clinical  pictures  is  necessary  for  a  proper  appre- 
ciation of  the  medicolegal  questions. 

Hysteria. — When  a  collision,  for  example,  occurs,  the  shock  and  the 
fright  into  which  the  passenger  is  thrown  are  sufficient  causes  to  disturb 
the  workings  of  the  entire  central  nervous  system  or  of  its  main  centers. 
Supposing  he  or  she  during  that  time  is  thrown  even  not  violently  against 
the  seat  of  the  car,  the  anticipation  alone  of  a  possible  severe  injury  or 
of  a  fatal  injury,  or  else  of  immediate  death,  is  capable  of  putting  out  of 
order  the  function  of  the  nervous  system.  During  the  first  few  days, 
or  even  weeks,  the  patient  still  dreads  that  his  life  is  in  danger. 

The  essential  features  of  hysteria  are: 

Sensory  Symptoms. — Hyperesthesia  in  areas  or  along  the  spine;  loss 
of  sensations  in  the  distant  segments  of  the  limbs  (glove-like  and  stocking- 
like anaesthesias);  anaesthesia  confined  to  an  entire  half  of  the  body  (hemi- 

548 


TRAUMATIC    NEUROSES    AND    PSYCHOSES  549 

anaesthesia) ;  anaesthesia  of  the  pharynx,  of  the  conjunctivae,  of  the  retina. 
The  latter  will  be  manifested  by  a  contraction  of  the  visual  field.  The 
special  senses  may  also  be  affected.  Sudden  partial  blindness  or  deafness 
(without  material  changes  in  the  eyes  or  ears) ,  vomiting  occurring  immedi- 
ately after  the  accident  and  continuing  for  several  days  or  weeks  without 
any  relation  to  the  food  and  with  an  excellent  appetite — all  these  symp- 
toms occur  quite  frequently. 

Motor  Symptoms. — Palsies,  contractures  confined  to  segments  of  limbs, 
are  not  infrequent,  but  they  are  never  accompanied  by  disturbances 
of  reflexes  usually  seen  in  organic  nervous  diseases.  The  absence  or  ex- 
aggeration of  knee-jerks,  toe  phenomena,  muscular  atrophies  with  reac- 
tions of  degeneration  accompany  organic  but  not  hysterical  palsies. 

The  function  of  the  viscera  may  also  be  disturbed.  Hys  terical  aphonia, 
anuria,  polyuria,  retention  of  urine,  are  well  known  phenomena.  In  a 
case  published  by  me  in  the  Medical  Record,  August,  1900,  a  woman  be- 
came anuric  after  an  emotion. 

Psychic  symptoms  are  quite  frequent.  Temporary  amnesia,  capri- 
ciousness,  inconsistencies  in  the  ideas  and  conduct,  dissociation  of  per- 
sonality, hallucinations  are  possible  occurrences  in  hysteria.  Hysterical 
subjects  are  easily  influenced  to  change  their  thoughts,  to  do  certain  acts, 
to  acquire  certain  sensations,  to  execute  or  to  adopt  certain  motor  phe- 
nomena. Suggestion  or  auto-suggestion  plays  an  enormous  role.  I  have 
also  seen  cases  of  hysterical  paroxysms  following  railroad  accidents.  A 
young  girl  of  seventeen,  while  lying  in  bed,  contorted  herself,  assumed  the 
position  of  opisthotonos,  was  animated  with  a  generalized  tremor,  so  that 
she  was  unable  to  utter  a  word  distinctly. 

These  are  the  characteristic  manifestations  in  the  motor,  sensory, 
and  psychic  spheres  of  a  hysterical  individual.  Of  course,  not  all  of 
them  are  simultaneously  found  in  every  case,  but  when  some  symptoms 
are  present  in  the  form  and  intensity  as  described  above,  the  diagnosis  of 
hysteria  can  be  made  without  hesitation. 

Neurasthenia. — This  neurosis  frequently  follows  accidents.  Its  main 
features  are:  physical  exhaustion  and  undue  irritability,  so  that  the  patient 
cannot  stand  the  least  contradiction  or  annoyance.  The  neurasthenic 
feels  fatigue  upon  the  least  exhaustion.  Mental  processes  are  also  slug- 
gish; the  least  mental  effort  disables  him  from  continuing  his  work.  The 
patient  complains  also  of  backache,  headache,  and  insomnia.  The 
symptoms  are  mainly  subjective.  The  objective  symptoms  are  very  few; 
tremor  of  the  hands,  increased  reflexes,  cold,  clammy  skin,  and  the  special 
facies.  The  latter  is  that  of  a  tired  and  depressed  individual  and  one  full 
of  anxiety. 


550  TEAUMATIC    NEUROSES    AND    PSYCHOSES 

Chorea,  with  its  irregular  and  incoherent  muscular  movements  rapidly 
following  one  another,  is  too  well  known  to  dwell  upon.  It  does  not 
frequently  follow  accidents.  Within  the  last  three  years  I  have  seen  only 
two  cases,  and  then  there  was  a  history  of  previous  attacks.  The  shock 
of  the  collision  was  immediately  followed  by  a  new  attack  of  chorea. 

The  same  remarks  can  be  made  about  Paralysis  agitans.  In  one  case 
of  a  middle-aged  woman  the  onset  of  the  shaking  palsy  was  traced  to 
twenty-four  hours  after  a  trolley  accident.  In  two  other  cases  (a  man  and 
a  woman)  the  shock  of  the  accident  was  the  direct  cause  of  aggravation 
of  the  previously  existing  symptoms,  which  from  that  time  kept  on  increas- 
ing in  intensity.  As  it  is  well  known,  the  disease  is  characterized  by  a 
continuous  tremor  affecting  mainly  one  or  both  hands  when  the  latter 
are  at  rest,  and  by  a  fixed  attitude  of  the  body,  mask-like  expression  of 
the  face. 

Amnesia. — This  term  is  applied  in  practice  to  an  acquired  diminution 
or  loss  of  memory.  There  are  two  classes  of  amnesias:  functional  and 
organic.  The  characteristic  feature  of  organic  amnesia  consists  of  its 
permanency  and  progressive  evolution.  Here,  by  virtue  of  an  organic 
alteration  of  nervous  elements,  the  memory  for  recent  events  is  first 
affected,  as  new  impressions  can  no  more  be  associated  and  preserved. 
The  gradually  old  intellectual  acquisitions  become  effaced. 

Functional  amnesia  is  due  to  the  profound  emotional  shock  caused 
by  trauma,  viz.  psychic  trauma.  The  sudden  commotion  of  the  nervous 
system  thus  created  disturbs  the  mechanism  of  association  of  ideas  with 
regard  to  time  and  place  and  produces  amnesia.  Functional  amnesia 
presents  by  far  more  variations  than  organic.  It  may  concern  only  a 
certain  group  of  ideas;  it  may  be  general,  in  which  all  past  events  are  lost; 
it  may  be  partial,  as  in  cases  of  double  personality,  a  striking  example 
of  which  was  reported  by  me  in  the  American  Journal  of  the  Medical 
Sciences,  1906;  it  may  be  localized,  when  it  concerns  only  a  certain  princi- 
pal fact  of  life.  Sometimes,  in  addition  to  the  latter,  a  certain  period 
of  time  immediately  preceding  it  is  forgotten.  We  then  speak  of  retro- 
grade amnesia.  When  the  period  of  time  following  the  principal  event 
is  forgotton,  there  is  an  anterograde  amnesia.  These  two  forms  may  be 
combined.  Within  the  last  three  years  I  have  seen  two  cases  of  retro- 
anterograde  amnesia  which  followed  a  fall  from  a  trolley  car  in  which  the 
patients  struck  their  heads  against  the  ground.  In  one  of  them  there  was 
loss  of  consciousness;  the  patients  were  confused  for  only  ten  minutes. 
A  careful  examination  revealed  in  both  cases  no  symptoms  of  an  organic 
lesion.  Subsequent  events  showed  the  diagnosis  in  ,both  cases  to  be  cor- 
rect, as  the  patients  made  an  uneventful  recovery. 


TRAUMATIC    NEUROSES    AND    PSYCHOSES  55 1 

Medicolegal  Considerations. — In  the  presence  of  an  individual  who, 
having  sustained  a  trauma,  complains  of  disturbances  of  a  nervous  or 
psychic  order,  the  following  medical  problems  must  be  solved:  (i)  Is  the 
alleged  disorder  genuine  or  simulated,  and  what  is  its  nature?  (2)  Should 
the  symptoms  observed  be  attributed  to  the  traumatism?  (3)  If  the  latter 
is  correct,  what  is  the  degree  of  incapacity  for  work,  what  are  the  prognosis, 
duration,  and  termination  of  the  malady? 

1.  The  determination  of  simulation  is  as  a  rule  not  difficult.  When 
an  affection  is  characterized  by  objective  signs,  they  will  be  easily  recog- 
nized. It  is  absolutely  impossible  for  anyone  to  simulate  an  anaesthetic 
pharynx,  and  anaesthetic  conjunctiva,  a  contraction  of  the  visual  field, 
a  genuine  hemianesthesia,  a  genuine  plus  reflex.  These  stigmata  taken 
together  constitute  the  typical  picture  of  hysteria.  This  malady  is  a 
well-defined  morbid  entity,  which,  except  in  rare  cases,  cannot  be  con- 
founded with  any  other  nervous  affection.  It  is  true  that  an  hysterical 
paroxysm  with  its  screaming,  laughing,  and  various  motor  and  psychic 
phenomena,  can  be  to  a  certain  extent  simulated,  but  an  experienced 
physician,  familiar  with  the  disease,  will  have  no  difficulty  in  recognizing 
it.  The  psychic  symptoms  of  hysteria  are  not  ordinary  symptoms;  they 
present  a  special  physiognomy,  the  main  points  of  which  can  never  be 
guessed  by  the  simulator.  It  is  the  entire  picture  of  the  attack  that  should 
be  taken  into  consideration,  not  individual  elements. 

To  illustrate  to  what  errors  a  non-f  amiliarity  with  the  disease  may  lead, 
the  following  is  a  case  in  point.  An  attorney  requested  me  to  examine 
one  of  his  clients,  who  claimed  to  have  developed  a  hemiplegia  after  a 
broken  trolley  wire  struck  him.  The  physician  in  charge  pronounced  it 
an  apoplectic  stroke  (there  was  a  partial  loss  of  consciousness)  caused  by 
the  electrical  current  of  the  wire.  The  patient  presented  a  limping  of  his 
left  leg  and  some  inability  to  use  the  left  arm.  The  condition  persisted 
almost  two  months.  Examination  showed  a  state  of  affairs  totally  differ- 
ent from  typical  hemiplegia  which  is  due  to  a  brain  lesion.  The  knee-jerk 
was  not  exaggerated,  and  the  abnormal  reflexes  (toe  phenomenon,  ankle- 
clonus)  usually  found  in  such  cases  were  absent.  The  entire  left  side  was 
also  wholly  anaesthetic.  It  was  unquestionably  a  case  of  hysteria.  The 
case  was  settled  out  of  court,  and  the  patient  made  a  complete  recovery 
shortly  after  he  got  his  money.  In  fact,  I  cured  him  with  a  few  seances  of 
a  strong  suggestive  static  breeze. 

A  woman  was  in  a  slight  railroad  accident.  She  was  only  thrown  on 
the  floor  of  the  car  and  there  was  no  surgical  injury.  When  picked  up  she 
could  not  hold  herself  in  an  erect  position  nor  could  she  walk.  The  legs 
gave  way  under  her.     The  condition  was  pronounced  grave  and  a  diagnosis 


552  TRAUMATIC    NEUROSES    AND    PSYCHOSES 

of  complete  paraplegia  due  to  a  hemorrhage  in  the  cord  was  made.  I  saw 
the  patient  since  then  on  several  occasions.  There  was  no  paralysis,  as 
she  could  move  the  limbs  easily  while  in  bed,  but  could  not  use  them  when 
standing  or  walking.  The  reflexes  were  normal,  sensations  normal,  and  the 
sphincters  intact.  It  was  a  case  of  hysterical  astasia-abasia,  from  which 
she  fully  recovered  after  a  course  of  rest  treatment  with  good  feeding. 

It  is  highly  important  to  always  bear  in  mind  the  possibility  of 
suggestion  during  an  examination.  Suggestion  is  also  one  of  the  causes  of 
hysteria.  The  examination  therefore  must  be  conducted  very  carefully. 
Sensations  should  be  investigated  by  having  the  patient  blindfolded.  No 
question  should  be  asked  of  her  while  the  test  is  being  made  with  a  pin 
over  various  areas  of  the  skin.  There  is  no  doubt  that  she  will  make  some 
defense  movement  if  the  prick  causes  pain.  Or  else  the  patient  may  be 
asked  to  say  "yes"  the  moment  she  feels  pain  or  the  touch  of  an  object. 
No  mention  should  be  made  to  the  patient  of  possibilities  of  loss  of  power 
in  one  or  more  limbs  or  segment  of  a  limb,  of  loss  of  memory,  of  a  grave 
outlook,  etc.  Various  disturbances  of  function  in  the  motor,  sensory, 
vaso-motor,  vegetative  and  psychic  spheres  may  be  suggested  to  a  hys- 
terical patient  with  great  facility. 

Far  more  difficulty  is  encountered  in  the  medicolegal  consideration  of 
neurasthenia.  This  malady  is  characterized  almost  exclusively  by  sub- 
jective manifestations.  When  a  patient,  having  met  with  an  accident, 
complains  of  fatigue,  backache,  headache,  insomnia  and  irritability,  there 
are  no  means  of  verifying  the  presence  or  absence  of  each  of  these 
symptoms. 

Nevertheless,  when  the  disease  is  pronounced,  the  general  aspect  of 
the  individual  will  aid  in  forming  an  opinion  as  to  the  veracity  of  his 
complaints.  There  is  usually  loss  in  weight,  loss  in  strength,  pallor  of 
the  face,  depression,  cold  and  clammy  skin,  tremor.  When  the  case  is 
mild,  the  latter  symptoms  are  absent,  and  then  one  has  to  rely  upon  the 
first  series  of  subjective  disturbances.  I  have  seen  time  and  again  that, 
in  spite  of  an  enormous  shock  following  for  example  a  collision  or  a  fall 
from  a  height,  only  a  few  subjective  symptoms  would  be  complained  of. 
In  such  cases  the  expert's  opinion  before  the  jury  and  court  should  be 
expressed  thus:  "If  the  subjective  symptoms  the  plaintiff  claims  to  suffer 
from  are  correct,  he  is  neurasthenic;  but  I  have  no  means  of  verifying 
them." 

In  such  a  manner  justice  is  done  to  both  sides,  defendant  and  plaintiff. 
The  expert's  role,  I  believe,  is  to  present  medical  facts  and  explain  their 
meaning  to  a  lay  jury.  The  decision  as  to  the  relation  of  these  symptoms 
to  the  accident  is  left  entirely  to  the  impartial  judgment  of  the  twelve  men. 


TRAUMATIC    NEUROSES    AND    PSYCHOSES  553 

In  the  majority  of  traumatic  cases  hysteria  and  neurasthenia  are 
combined.  This  facilitates  considerably  the  problem,  as  objective  signs 
are  almost  always  present,  and  if  there  is  a  certain  hesitancy  in  the  mind 
of  the  physician  in  accepting  the  subjective  symptoms,  due  credit  should 
be  given  to  the  objective  phenomena  of  the  victim  of  the  accident. 

Chorea  and  Paralysis  agitans  developed  after  a  trauma  present  no 
difficulty  in  being  recognized.  Simulation  in  such  cases  is  an  impossibility 
and  the  objective  symptoms  are  too  evident  to  be  contested. 

Considerable  difficulty  will  be  encountered  when  amnesia  is  the  only 
symptom  produced  by  the  accident.  If  the  loss  of  memory  is  associated 
with  hysteria,  the  symptoms  of  the  latter  will  render  sufficient  aid,  but 
when  hysterical  stigmata  are  absent,  the  difficulty  becomes  very  great  in 
deciding  the  question  whether  the  amnesia  is  genuine  or  not.  In  such 
cases  the  examination  should  be  repeated,  the  individual  must  be  tested 
with  great  perseverance  and  patience,  he  must  be  questioned  as  closely  as 
possible  and  for  a  prolonged  period  of  time.  Great  skill  and  tact  are 
necessary  in  investigations  of  this  sort,  and  in  some  cases  one  may  arrive 
at  a  positive  opinion. 

2.  The  second  medicolegal  problem  is,  as  I  said  above,  to  determine 
whether  the  maladies  discovered  in  an  individual  are  attributable  directly 
to  the  accident.  While  a  shock,  physical  or  mental,  is  a  frequent  cause 
of  functional  nervous  diseases,  one  cannot  nevertheless  be  affirmative  in 
a  given  case  unless  the  state  of  health  prior  to  the  accident  is  known  to  the 
examiner.  Hysteria,  neurasthenia,  chorea,  or  paralysis  agitans  may  have 
existed  before  the  plaintiff  sustained  the  shock.  It  should,  however,  not 
be  forgotten  that  if  any  of  those  affections  existed  before,  the  newly  sus- 
tained shock  will  aggravate  it.  A  person,  for  example,  is  supposed  to  be 
in  the  process  of  recovery  from  a  neurosis.  Should  he  at  that  time  sustain 
an  injury,  the  primary  disease  is  likely  to  return  and  present  itself  in  a 
more  pronounced  form  than  in  its  first  attack. 

Individual  predisposition  should  also  be  taken  into  consideration  in 
giving  an  account  before  court  and  jury.  Neuropathic  individuals  are 
more  apt  to  suffer  from  any  of  these  neuroses  than  normal  individuals. 
They  are  predisposed,  and  the  least  shock,  a  comparatively  slight  trauma 
is  sufficient  to  disturb  the  workings  of  their  nervous  system  with  the 
greatest  facility.  Individuals  whose  vitality,  and  therefore  resisting  power, 
has  been  lowered  following  a  protracted  infectious  disease,  lead  intoxica- 
tion, alcoholism,  syphilis,  are  inclined  to  respond  with  unusual  prompt- 
ness to  the  effects  of  a  shock.  The  neuroses  will  find  a  fertile  soil  for  their 
development.  It  is  consequently  important  to  surround  one's  self  with 
this  precaution  in  giving  an  estimate  of  the  degree  of  the  individual's 


554  TRAUMATIC    NEUROSES    AND    PSYCHOSES 

suffering.  Not  only  the  neuropathic  tendency  or  the  existence  of  a  previ- 
ous serious  disease  of  the  victim  of  the  accident  must  be  known  to  the  phy- 
sician, but  also  the  knowledge  of  the  previous  conduct  and  habits  of  the 
injured  individual  is  absolutely  necessary.  Sexual  or  alcoholic  excesses, 
sleepless  nights,  irregular  moc^e  of  living  in  every  respect — are  all  predis- 
posing elements  for  neuroses.  The  question  of  just  compensation  con- 
sequently can  be  decided  after  a  complete  knowledge  of  all  factors  con- 
cerned in  any  given  case. 

In  view  of  the  fact  that  trauma  may  actually  incapacitate  an  individual 
for  a  long  time,  the  possibility  of  malingering  should  never  be  overlooked. 
In  cases  in  which  the  symptoms  are  chiefly  subjective  there  may  sometimes 
be  some  difficulty  in  differentiating  true  neurasthenia  from  malingering. 
Here  the  knowledge  of  the  victim's  previous  habits  and  conduct  is  essential. 
However  a  malingerer  usually  exaggerates  especially  objective  mani- 
festations, such  as  paralysis  or  hyperesthesias.  He  will  often  speak  of 
loss  of  consciousness  during  the  accident  and  at  the  same  time  give  a 
correct  account  of  all  happenings.  Such  an  individual  should  be  thor- 
oughly scrutinized  and  with  a  skillful  manner  of  questioning  and  of  elicit- 
ing the  objective  symptoms  there  will  be  no  great  difficulty  in  the  majority 
of  cases  to  arrive  at  a  proper  diagnosis. 

3.  The  last  proposition  of  our  problem  is  to  determine  the  degree  of 
incapacity  caused  by  the  accident,  the  consequences,  duration,  and  ter- 
mination of  the  latter. 

Hysteria  and  neurasthenia  are  not  synonymous  of  simple  nervousness, 
as  some  pretend.  They  are  well-defined  diseases  of  the  nervous  system. 
They  do  incapacitate  for  mental  or  physical  work,  but  only  to  a  certain 
extent.'  A  neurasthenic  with  his  chronic  physical  and  mental  fatigue 
is  certainly  not  able  to  accomplish  much.  The  disease  presents  variations 
in  its  intensity.  If  the  symptoms  are  marked,  the  patient  must  go  to  bed, 
as  rest  is  the  most  essential  element  of  the  treatment.  Then  there  is  no 
question  of  work.  When  the  case  is  mild,  and  the  fatigue  is  not  particu- 
larly marked,  and  the  backache  with  the  headache  only  occasionally  dis- 
turb the  patient,  a  certain  amount  of  work  can  be  done  by  the  patient,  and 
with  the  proper  regulation  of  the  patient's  mode  of  living  he  can  be  made 
to  feel  quite  comfortable. 

Hysterical  patients  are  not  very  much  disturbed  in  their  daily  life 
when  the  psychic  symptoms  are  not  present.  One  may  bear  the  anaes- 
thesias, hyperesthesias,  and  tremor,  also  contraction  of  the  visual  field 
without  being  particularly  annoyed.  But,  as  a  rule,  there  are  psychic 
phenomena,  viz.  great  emotionality,  irritability,  crying  spells,  restlessness, 
impressionableness,  etc.     Such  patients  are  unfit  for  work.     Their  mental 


TRAUMATIC    NEUROSES    AND    PSYCHOSES  555 

concentration  and  application  are  of  a  very  short  duration.  Whatever 
they  commence,  they  are  unable  to  finish.  Their  association  of  ideas  is 
incomplete  because  of  instability  and  want  of  depth  of  mental  processes. 
For  this  very  reason  good  work  cannot  be  expected  from  those  in  whom 
psychic  disturbances  are  marked.  Even  in  those  cases  which  are  appar- 
ently free  from  mental  symptoms,  the  least  emotion  or  undue  exertion 
will  bring  forward  the  above  psychic  manifestations  so  characteristic  of 
hysteria,  because  the  nature  of  the  disease  predisposes  them  to  an  unusual 
responsiveness.  The  degree  of  incapacity  will  depend  upon  whether  the 
psychic  symptoms  are  present  or  not,  also  upon  the  intensity  of  the 
latter. 

Chorea  and  paralysis  agitans  incapacitate  for  physical  work  consid- 
erably, but  only  partially  for  mental  work. 

When  amnesia  occurs,  a  disability  will  ensue  if  it  is  of  a  generalized 
character.  If  it  concerns  only  a  certain  past  period  of  life  (retrograde  or 
anterograde  form),  there  is  no  disability  whatever.  The  individual  is 
perfectly  able  to  earn  a  living,  as  the  memory  is  good  for  all  events  except 
that  one. 

A  proper  discrimination  as  to  degree  of  incapacity  in  all  the  neuroses 
is  always  possible  when  the  above  elements  are  taken  into  consideration. 

Not  infrequently  we  are  asked  about  the  future  of  the  victims  of 
accidents.  My  answer  is  that  while  the  neuroses  are  curable  affections 
they  nevertheless  may  last  an  indefinite  time.  The  results  depend  upon 
many  circumstances,  viz.  treatment,  surroundings  of  the  patient,  previous 
general  health,  ability  or  inability  to  carry  out  certain  instructions, 
outside  influences,  and  finally  the  individual  make-up.  In  exceptional 
cases  the  disease  may  last  for  years  in  spite  of  treatment.  It  should  not 
be  forgotten  that  recurrences  are  possible,  and  in  fact  are  not  infrequent. 
In  the  majority  of  cases  the  patients  make  a  good  recovery.  The  latter 
is  possible  in  hysteria,  neurasthenia,  chorea,  and  amnesia,  but  not  in 
paralysis  agitans. 

Psychoses.— Mental  disorders  present  a  very  important  chapter  from 
a  medicolegal  standpoint.  A  shock  caused  by  an  accident  is  likely  to 
produce  mental  disturbances,  but  between  the  latter  and  distinct,  well- 
defined,  mental  affections  there  is  a  great  difference. 

Hysteria  is,  properly  speaking,  a  psychic  disturbance,  and  its  mental 
phenomena  described  above  belong  by  right  to  this  chapter.  They  have 
been  sufficiently  emphasized  on  the  preceding  pages.  Amnesia  is  an- 
other psychic  phenomenon.  It  is  also  f amilar  to  us.  When  after  a  trauma 
concussion  of  the  brain  is  suspected,  the  period  of  unconsciouness  may  be 
followed  by  some  mental  symptoms,  viz.  dullness  and  sometimes  con- 


556  TRAUMATIC    NEUROSES    AND    PSYCHOSES 

fusion,  inability  to  recall  the  circumstances  of  the  accident  (retrograde, 
anterograde  or  retroanterograde  amnesia),  inability  to  exercise  the  power 
of  attention;  disturbance  of  judgment,  marked  indecision  in  undertakings. 
All  these  symptoms  are  aggravated  by  insomnia  which  is  usually  present 
in  such  cases.  These  patients  are  ordinarily  conscious  of  their  mental 
deficiency  and  we  observe  that  anxiety  and  worry  over  their  condition 
and  their  future  are  added  to  the  above  disorder. 

When  the  immediate  symptoms  of  brain  injury  are  severe,  there  is 
usually  a  cerebral  hemorrhage  or  fracture  of  the  skull.  A  cerebral  con- 
tusion, followed  perhaps  by  minute  disseminated  hemorrhages,  is  apt  to 
be  followed  by  mental  symptoms,  such  as  vertigo,  delirium,  confusion, 
loss  of  memory,  and  coma.  In  such  cases  the  patient  either  dies  or  gradu- 
ally recovers  his  mental  faculties.  Korsakoff's  psychosis  may  be  the 
result  of  a  severe  injury.  We  then  have  a  marked  confusion,  illusions  of 
identity,  disorientation  of  space  and  time,  loss  of  memory  for  recent 
events  and  fabrication.  Alcoholism  is  usually  the  underlying  cause  (see 
for  details  page  347). 

In  infancy  and  childhood  the  condition  is  somewhat  different.  When 
a  child  in  the  process  of  development  undergoes  the  effect  of  a  severe 
cerebral  injury,  the  result  may  be  very  serious.  If  epilepsy  follows,  there 
is  no  doubt  that  the  mental  growth  will  be  interfered  with.  Faulty 
cerebral  development  may  follow  a  grave  injury  to  the  head,  irrespective 
of  epilepsy.  The  prognosis,  therefore,  is  guarded  when  arrest  of  intel- 
lectual development  is  observed  shortly  after  a  cranial  injury. 

The  relation  of  paresis  to  traumata  is  a  question  of  great  importance. 
There  are  some  alienists  of  note  who  believe  that  an  injury  is  apt  to  develop 
paresis,  others  find  no  relation  between  the  two.  Paresis  is  an  incurable 
disease;  it  is  one  of  the  most  serious  of  mental  affections.  If  its  symptoms 
are  noticed  first  after  an  accident,  one  "is  naturally  apt  to  attribute  them 
to  the  latter.  At  the  Congress  of  the  French  Neurologists  and  Alienists 
in  1906  this  question  was  the  subject  of  a  special  report,  so  important  it 
was  considered.  It  was  discussed  at  length  by  the  most  brilliant  minds. 
It  is  frequently  brought  forward  as  of  a  special  import,  as  courts  and  law- 
yers are  waiting  for  our  decision. 

In  the  light  of  our  present  knowledge  concerning  especially  the  com- 
plement fixation  test  it  may  be  safely  said  that  paresis  is  not  and  cannot 
be  caused  by  an  accident.  It  is  a  mental  disease  of  a  slow,  but  progressive 
evolution,  and  characterized  pathologically  by  a  gradual  degeneration 
of  cerebral  tissue.  If  its  symptoms  become  sometimes  conspicuous  after 
a  trauma,  is  it  reasonable  to  suppose  that  a  cerebral  degeneration  sets  in 
immediately  and  at  once  produces  the  symptoms  the  development  of 


EPILEPSY   IN   RELATION    TO    TRAUMA  557 

which  require  a  long  period  of  morbid  changes  in  coritcal  cells?  In  my 
judgment  the  disease  existed  before  the  accident,  and  the  latter  served 
only  as  an  exciting  cause  for  its  more  rapid  development.  Medical 
records  are  abundant  with  examples  of  this  nature  in  other  affections. 

The  mental  manifestations  of  paresis,  in  its  expansive  or  depressive 
forms,  the  physical  signs,  such  as  irregular  and  unequal  pupils,  the  dis- 
turbance of  the  pupillary  reflexes,  characteristic  speech,  tremor  of  the 
tongue  and  hands,  changes  in  the  reflexes  (loss  or  increase),  apoplectiform 
or  epileptiform  seizures — it  is  not  conceivable  that  these  symptoms  will 
explode  shortly  after  an  accident  without  being  in  existence  prior  to  the 
accident.  We  are  now  in  possession  of  bio-chemical  means  to  determine 
the  nature  of  paresis,  so  that  in  doubtful  cases  they  render  the  most 
valuable  aid  in  diagnosis.  Positive  Wassermann  reaction  of  blood  serum 
and  cerebro-spinal  fluid,  increase  of  albumen  in  the  latter,  finally 
lymphocytosis — are  all  pathognomonic  of  paresis.  The  following 
peculiarities  concerning  these  reactions  are  worth  mentioning,  (i)  If 
the  blood  serum  does  not  give  a  positive  reaction,  the  cerebro-spinal 
fluid  will  be  negative.  If  the  cerebro-spinal  fluid  gives  a  positive  result, 
the  blood  serum  does  the  same.  (2)  If  the  serum  gives  a  positive  result, 
but  the  cerebro-spinal  fluid  a  negative,  the  disease  is  not  paresis,  but 
cerebro-spinal  syphilis.  (3)  The  examination  of  the  cerebro-spinal  fluid 
is  more  important  in  paresis  than  that  of  blood  serum.  (4)  Mercury, 
iodides,  salvarsan  may  render  the  Wassermann  reaction  negative  for  a 
certain  time  . 

If  after  a  trauma  these  reactions  are  found  to  be  present,  there  can  be 
no  doubt  that  paresis  existed  before  the  trauma.  The  latter  per  se  cannot 
create  any  of  the  four  reactions  mentioned. 

EPILEPSY  IN  RELATION  TO  TRAUMA 

It  is  a  well-known  fact  that  trauma  may  cause  epilepsy.  A  fracture 
of  the  skull  may  occur  without  any  material  evidence  on  the  scalp.  In 
such  cases  the  inner  table  alone  may  be  injured  and  a  fragment  may  press 
directly  on  the  cortical  layer  of  the  motor  area.  In  other  cases  there  may 
be  some  cicatricial  tissue  with  thickening  of  the  meninges  producing  an 
irritation  of  the  motor  area.  In  still  other  cases  no  appreciable  lesion 
of  the  motor  cortex  had  been  found  and  still  epilepsy  developed  after 
a  trauma  (Raymond).  According  to  Kocher  and  Pitres  the  cause  of 
epilepsy  in  such  cases  is  the  increased  pressure  of  the  cerebro-spinal 
fluid.  In  every  case  of  traumatic  epilepsy  one  must  hear  in  mind  the 
possibility  of  preexisting  epilepsy  or  of  the  individual  being  predisposed 


558  EPILEPSY    IN   RELATION    TO    TRAUMA 

to  it  through  alcoholism  or  syphilis.  In  such  cases  the  trauma  plays  the 
role  of  a  contributory  factor.  The  following  case  of  epilepsy  is  of  interest 
fron  a  medicolegal  standpoint. 

The  man  I  was  called  upon  to  examine  fell  off  a  ladder  while  working 
at  a  building.  He  struck  his  head  and  became  unconscious.  In  the 
evening  of  the  same  day  he  had  an  epileptiform  seizure  with  generalized 
convulsive  movements.  He  was  taken  away  from  work,  kept  at  home, 
and  treated  for  epilepsy.  Since  then  his  mental  faculties  became  ob- 
tunded.  His  memory  began  to  fail.  He  was  unable  to  give  an  account 
of  himself.  He  would  make  grave  mistakes.  Would  ask  for  a  fork 
when  he  wanted  a  knife;  would  forget  his  wife's  name.  He  would  try 
to  get  in  his  neighbor's  house  instead  of  in  his  own.  He  also  had  out- 
breaks of  extreme  furor,  in  which  he  would  use  profane  language,  and 
even  strike,  so  that  the  lives  of  his  relatives  became  endangered.  After 
the  explosion  of  this  passion  subsided,  he  had  no  recollection  of  what 
occurred. 

When  I  saw  him  months  later  he  presented  marked  tremor  of  lips, 
increased  reflexes,  pronounced  confusion,  and  a  vague  expression  of  the 
eyes  with  dilated  pupils.  He  could  not  give  me  an  explanation  of  those 
outbreaks  of  passion,  of  which  he  had  no  recollection.  The  patient  had 
evidently  been  suffering  from  epileptic  dementia.  The  attacks  he  pre- 
sented were  psychic  in  form,  but  of  epileptic  nature. 

The  question  arose  in  court  whether  the  trauma  was  the  direct  cause 
of  his  mental  condition.  A  very  careful  search  into  the  man's  previous, 
life,  with  data  obtained  from  his  wife,  who  was  separated  from  him  before 
the  accident,  also  from  his  relatives  and  friends,  revealed  the  fact  that 
while  the  man  never  had  a  seizure  prior  to  the  trauma,  and  was  mentally 
clear  as  he  worked  in  the  same  place  for  seven  years,  he  nevertheless  had 
a  syphilitic  infection  and  used  alcohol  to  an  unusual  excess. 

My  opinion,  based  upon  all  the  facts  of  the  case,  was  formulated  as 
follows:  "The  trauma  was  an  exciting  cause  to  the  epilepsy  with  its 
dementia,  but  the  patient  was  predisposed  to  the  disease.  Any  other 
cause  would  have  produced  the  same  condition.  It  is  also  possible  that 
the  mental  affection  would  have  developed  without  any  apparent  cause, 
as  chronic  alcoholism  and  syphilis  are  sufficient  etiological  factors  in 
epilepsy." 

An  accident  may  be  the  exciting  cause  of  a  delirium  or  a  delirium 
tremens  in  an  individual  prof oundly  intoxicated  with  alcohol.  Similarly 
I  have  seen  a  confusional  state  developed  in  individuals  with  a  previous 
alcoholic  history.  A  thorough  investigation  of  the  personal  antecedents, 
is  absolutely  indispensable  in  cases  of  this  category. 


EPILEPSY    IN    RELATION    TO    TRAUMA 


559 


The  previous  study  of  trauma  in  relation  to  nervous  and  mental  dis- 
orders leads  to  the  following  conclusion.  In  cases  of  railway  or  other 
injuries  caused  by  neglect  of  those  who  have  in  charge  the  management  of 
transportation  cars,  it  is  no  more  than  just  that  the  injured  person  should 
be  compensated  for  disability.  On  the  other  hand  simulation  or  exaggera- 
tion of  incapacity  should  be  condemned.  The  physician  is  indispensable 
to  the  law.  In  the  name  of  justice  he  must  be  invariably  reserved  in 
his  statements.  His  opinion  must  be  formed  after  a  thorough  study  of 
each  individual  case.  He  must  not  forget  that,  while  some  severe  trau- 
matisms may  produce  mild  symptoms,  some  insignificant  traumata  may 
cause  marked  disturbances  of  the  nervous  system.  The  degree  of  the 
disability  and  the  prognosis  of  the  affection  vary  in  each  individual  case. 
The  recognition  of  the  affection,  the  recognition  of  the  influence  of  the 
accident  upon  its  manifestations,  finally  the  discrimination  of.  a  genuine 
malady  from  a  simulated  one — all  these  elements  can  be  acquired  only 
when  the  physician  is  properly  prepared. 


CHAPTER  XXVII 

DISEASES  OF  THE  SYMPATHETIC  NERVOUS  SYSTEM 
TROPHONEUROSES.    ANGIONEUROSES 
Exophthalmic  Goiter 
(Grave's  or  Basedow's  Disease) 

This  affection  is  characterized  by  four  cardinal  symptoms:  enlarge- 
ment of  the  thyroid  gland,  exophthalmos,  tachycardia  and  tremor.     In 

the  majority  of  cases  they  are  all  present. 

Symptoms. — In  typical  cases  the  disease  develops  slowly.  In  excep- 
tional cases  all  or  almost  all  the  characteristic  symptoms  appear  suddenly 
or  rapidly. 

i.  The  most  constant  and  earliest  symptom  is  tachycardia.  The 
least  muscular  effort  or  emotion  brings  on  an  attack  of  palpitation  which 
may  be  so  pronounced  that  the  heart  beat  can  be  perceived  at  a  distance. 
Arhythmia  with  signs  of  asystoly  (cyanosis,  oedema,  vertigo)  is  observed 
sometimes.  Precordial  pain  is  frequently  present.  The  carotid  arteries 
are  full  and  beat  violently.  The  pulse  is  about  120  and  more  per  minute. 
Examination  of  the  heart  shows  nothing  abnormal  in  most  cases,  although 
in  advanced  stages  it  may  become  dilated  and  a  mitral  insufficiency 
develops. 

2.  Gradually  the  neck  begins  to  get  large  and  a  goiter  develops. 
The  enlargement  may  affect  the  entire  thyroid  gland  or  only  one  lobe. 
The  goiter  is  soft  and  vascular.  A  systolic  bruit  is  often  heard  on  auscul- 
tation. The  goiter  is  not  painful,  but  when  it  increases  rapidly,  it  pro- 
duces symptoms  of  compression  and  threatening  suffocation. 

3.  Exophthalmos  is  very  frequent,  but  not  constant.  It  is  usually 
bilateral  and  occasionally  unilateral.  It  may  be  more  marked  on  one 
side  than  on  the  other.  The  protrusion  of  the  eye  globe  gives  the  face 
an  expression  of  fright  and  anger.  Closing  of  the  eyelids  is  impossible. 
The  exposure  of  the  conjunctiva  and  cornea  leads  to  inflammation  and 
ulceration.  Lachrymation  is  frequent.  The  palpebral  fissure  is  some- 
times very  wide  because  of  a  contraction  of  the  levator  palpebree  and  the 
patient  is  then  unable  to  wink.  This  is  Stelwag's  sign.  There  are  other 
signs  that  accompany  exophthalmos.    Von  Graefe's  sign  consists  of  an 

560 


EXOPHTHALMIC    GOITER 


56l 


inability  of  the  eyelid  to  follow  the  movements  of  the  eyeglobe;  when 
the  eye  is  in  the  act  of  moving  upward  or  downward,  there  is  a  delay 
in  the  movement  of  the  eyelid.  Mobius'  sign  consists  of  difficulty  of 
convergence. 

External  ophthalmoplegia  has  been  observed.     The  visual  acuity  is 
diminished.     Hyperaemia  of  the  retinal  blood  vessels  is  sometimes  noticed. 


/&L 

g^^ 

1 1 

- 

fjff:; 

t\ 

Fig.  161.— Exophthalmic  Goiter. 


The  eye-grounds  are  usually  normal.     Amblyopia,  diplopia,  are  very  rare 
occurrences. 

4.  Tremor  is  very  frequent  and  it  is  .particularly  significant  in  the  in- 
complete forms.  It  is  a  fine,  vibratory  tremor.  The  oscillations  are 
rapid  (eight  to  ten  per  second).  It  is  localized  or  generalized.  It  affects 
more  frequently  the  hands,  but  also  the  head,  trunk  and  the  lower  extremi- 
ties. It  is  present  during  rest,  but  more  marked  on  voluntary  acts  and 
emotion. 
36 


562  EXOPHTHALMIC    GOITER 

Besides  the  above  four  typical  signs  there  are  others  which  may  occur 
now  and  then  in  the  course  of  Grave's  disease. 

Motor  Symptoms. — They  are:  paresis  of  the  lower  extremities  or  of 
the  facial  muscles,  hemiplegia,  monoplegia,  but  they  are  all  transitory. 
Cramps,  contractures,  tetany,  epilepsy,  occur  occasionally.  The  reflexes 
are  usually  unaltered,  but  they  may  be  increased  or  decreased. 

Sensory  Symptoms. — Pain  of  a  neuralgic  character,  especially  in  the 
eyeglobes,  face,  neck,  also  in  the  precordial  region,  in  the  arms,  is  not 
infrequent. 

Vasomotor  and  Trophic  Disturbances. — Flushes  of  heat  in  the  head, 
profuse  perspiration,  slight  elevation  of  temperature  several  times  during 
the  day,  are  quite  commonly  observed. 

The  skin  may  be  the  seat  of  various  eruptions.  Urticaria,  oedema, 
pigmentation,  vitiligo,  occur  sometimes.  Association  of  Grave's  disease 
with  scleroderma  has  been  reported.  Falling  out  of  the  hair  is  not  in- 
frequent. Increase  of  galvanic  reaction  in  the  skin  and  various  tissues 
is  frequently  observed.  Nutritive  changes  are  present.  There  is  usually 
emaciation  and  general  weakness. 

Urinary  Symptoms. — Polyuria,  albuminuria,  glycosuria,  occur.  The 
first  is  quite  frequent. 

Digestive  Disturbances. — Anorexia  or  else  polydipsia,  vomiting,  diar- 
rhoea are  observed.    Excessive  salivation  and  j  aundice  are  rare  occurrences. 
Respiratory  Disturbances.— Dyspnoea,  dry  cough,  inability  to  execute 
forced  inspiration  (Bryson's  sign)  are  observed. 

Disturbed  Genital  Function.— Atrophy  of  the  organs  and  of  the 
mammary  glands,  impotence,  diminution  of  sexual  desire,  amenorrhcea, 
occur. 

Psychic  Disturbances.— Restlessness,  irritability,  instability,  changes 
from  gay  and  happy  mood  to  depression,  sadness,  and  anger  are 
frequently  present.  Insomnia  is  the  rule.  Hypochondriacal  ideas 
frequently  develop.  In  some  cases  delirium  and  confusion  with  hal- 
lucinations occur. 

Forms. — The  above  clinical  picture  is  that  of  typical  cases  of  ex- 
ophthalmic goiter.  There  are  cases  in  which  not  all  of  the  four  chief 
symptoms  are  present  (incomplete  forms).  Grave's  disease  without 
goiter  or  else  without  exophthalmos  is  not  very  rare.  Other  forms  are 
marked  by  a  slow  development  of  symptoms  (chronic).  Others  (acute) 
are  conspicuous  by  rapidity  of  development. 

Association  of  Grave's  disease  with  hysteria  is  frequent.  Some- 
times it  is  seen  with  epilepsy  and  occasionally  with  tetany,  chorea,  tabes, 
syringomyelia.     I  reported  a  case  of  exophthalmic  goiter  associated  with 


EXOPHTHALMIC    GOITER  563 

paralysis  agitans  {New  York  Med.  Jour.,  December  31,  1904).  Finally 
it  may  be  also  met  with  in  diabetes,  myxcedema  and  insanity. 

Course,  Duration,  Prognosis. — The  symptom-group  is  rarely  com- 
plete, but  the  phenomenon  which  is  never  wanting  and  which  appears 
at  the  onset  of  the  malady  is  the  cardiac  disturbance.  The  acute  and 
chronic  varieties  have  been  mentioned.  The  course  is  rarely  regular:  it 
varies  from  individual  to  individual.  Amelioration  and  aggravation  of 
the  symptoms  occur  frequently. 

In  the  majority  of  cases  the  duration  is  protracted.  It  may  last 
many  years  or  indefinitely.  Recoveries  rarely  occur.  The  prognosis 
depends  upon  the  severity  of  the  symptoms.  Anorexia,  diarrhoea,  al- 
buminuria, produce  cachexia  and  hasten  death.  Sometimes  death  comes 
on  very  rapidly.  Compression  of  the  trachea  by  the  goiter,  asystoly, 
may  be  the  cause  of  rapid  death.  In  some  cases  an  intercurrent  disease 
leads  promptly  to  a  fatal  termination.  Pulmonary  tuberculosis  is  not 
rare.  In  the  most  favorable  cases  some  traces  of  exophthalmos,  of  goiter, 
remain. 

Diagnosis. — The  typical  form  will  be  recognized  from  its  cardinal 
signs.  Difficulties  will  be  encountered  in  the  abnormal  or  incomplete 
forms.  In  such  cases  the  constant  presence  of  tachycardia  and  very 
frequent  presence  of  tremor  will  aid  in  tracing  the  affection.  Hysteria 
associated  with  chlorosis  may  simulate  Grave's  disease,  but  the  cardiac 
disturbance  in  the  chlorosis  is  not  as  continuous  as  in  exophthalmic  goiter. 

Simple  goiter  with  tachycardia  may  also  embarrass  the  diagnosis. 
It  is  the  general  picture  of  the  disease  that  should  always  be  taken  into 
consideration  while  making  a  diagnosis. 

Etiology. — A  neuropathic  heredity  can  be  traced  in  the  majority  of 
cases.  Acute  infectious  diseases  (typhoid  fever,  inflammatory  rheuma- 
tism, grippe,  scarlet  fever,  pertussis)  may  be  followed  by  Grave's  disease. 
Syphilis,  lead  intoxication,  tuberculosis,  ordinary  goiter,  are  all  predispos- 
ing factors. 

Among  the  exciting  causes  may  be  mentioned  violent  emotions, 
traumata,  excesses  (sexual  and  others),  pregnancy. 

Pathogenesis. — The  pathological  investigations  show  changes  in  the 
thyroid  gland,  thymus,  cervical  sympathetic  nerve  and  medulla.  The 
thyroid  gland  may  present  many  morbid  varieties  from  a  simple  conges- 
tion to  the  most  pronounced  lesions.  Generally  there  is  hypertrophy 
of  the  entire  gland,  of  one  lobe  or  of  a  portion  of  a  lobe.  The  vesicles 
are  increased  in  size,  the  epithelium  is  hypertrophied;  the  intravesicular 
colloidal  substance  is  usually  diminished  in  quantity;  in  the  parenchyma 
of  the  gland  there  are  lymphoid  masses  which  are  considered  character- 


564  EXOPHTHALMIC    GOITER 

istic  of  exophthalmic  goiter.  The  connective  tissue  of  the  gland  is 
abnormally  developed.  The  blood  vessels  are  dilated  and  their  walls  are 
thickened. 

The  same  condition  has  been  found  in  the  thymus.  , 

The  cervical  sympathetic  ganglia  and  nerve  have  also  been  found 
altered  (proliferation  of  connective  tissue,  multiplication  of  blood  vessels, 
atrophy  of  cells). 

In  the  medulla  dilatation  of  blood  vessels  and  hemorrhages,  atrophy 
of  the  restiform  bodies  have  been  reported  by  competent  observers. 

In  view  of  such  a  great  variety  of  pathological  changes  the  nature 
of  Grave's  disease  remains  as  yet  obscure. 

The  most  favorable  view  held  at  present  is  that  the  thyroid  gland 
is  the  only  cause  of  the  affection.  The  theory  is  as  follows.  The  function 
of  the  thyroid  gland  consists  of  extracting  from  the  system  a  toxic  product 
and  neutralizing  it  before  it  is  thrown  into  the  general  circulation.  The 
neutralization  is  performed  by  a  special  product  secreted  by  the  gland. 
In  Grave's  disease  there  is  an  excessive  secretion  of  the  gland  (hyper- 
thyroidization) .  The  toxic  product  thrown  into  the  circulation  irritates 
the  cervical  sympathetic  nerve,  which  then  produces  the  symptoms  of 
the  eyes,  of  the  heart  and  the  goiter.  This  theory  cannot  satisfactorily 
explain  why,  if  a  toxin  is  at  fault,  it  excites  only  the  cervical  sympathetic 
nerve  and  not  also  the  other  sympathetic  nerves.  It  also  fails  to  explain 
cases  with  enormous  exophthalmos  with  insignificant  thyroid  enlargement, 
cases  with  enormous  goiters  and  insignificant  exophthalmos,  cases  of 
unilateral  Grave's  disease. 

On  the  other  hand  there  is  a  number  of  cases  on  record  showing  that 
many  nervous  diseases  become  complicated  with  symptoms  of  Grave's 
disease  when  in  the  later  stages  bulbar  symptoms  make  their  appearance. 
Such  are  the  observations  on  tabes,  amyotrophic  lateral  sclerosis,  polioen- 
cephalitis, pachymeningitis  cervicalis,  syringomyelia.  Experimental  phys- 
iology (Filehne,  Bienfait,  Durdufi),  post-mortem  examinations  collected 
by  H.  Klein  {Deutsche  Zeitschrift  f.  Neurologie,  1904)  show  changes  in 
the  medulla  and  pons,  especially  hemorrhages. 

In  a  case  observed  by  me  (N.  Y.  Med.  Jour.,  1905)  a  woman  suddenly 
developed  a  paralysis  of  the  third,  fourth  and  sixth  nerves  unequally 
distributed  on  both  sides.  A  few  days  later  she  noticed  a  gradually 
coming-on  prominence  of  both  eyes.  A  week  later  a  goiter  with  tachy- 
cardia and  tremor  began  to  develop.  There  was  also  von  Graefe's  sign 
(Fig.  161). 

All  these  considerations  tend  to  prove  that  the  phenomena  of  Grave's 
disease  are  due  to  a  bulbar  disturbance:  the  vaso-dilators  of  the  head 


EXOPHTHALMIC    GOITER  565 

and  heart  which  have  their  deep  origin  in  the  medulla  and  situated  in 
the  cervical  sympathetic  nerve,  undergoing  irritation,  send  a  constant 
afflux  of  blood  to  the  thyroid  and  to  the  retrobulbar  vessels;  the  filaments 
going  to  the  heart  give  rise  to  tachycardia. 

Treatment. — The  first  indication  is  to  place  the  patient  in  a  con- 
dition of  keeping  his  nervous  system  free  from  emotions  or  shocks.  Rest, 
proper  hygiene,  regular  and  quiet  mode  of  living  and  removal  from  the 
usual  surroundings — better  in  the  country — are  all  beneficial  means. 
He  should  avoid  exertion,  climbing  stairs,  mountains.  The  tachycardia 
which  is  always  present,  and  which  is  distressing,  will  thus  be  ameliorated. 

All  stimulants,  including  tea  and  coffee,  the  use  of  tobacco,  must  be 
avoided.  Sexual  intercourse  is  forbidden.  Marriage  is  also  contra- 
indicated. 

The  diet  should  be  nutritious,  but  not  abundant.  Milk  is  an  ideal 
food  in  such  cases.  Constipation  is  to  be  avoided,  but  powerful  purga- 
tives should  not  be  administered. 

Internally  the  following  remedies  can  be  tried,  but  not  much  reliance 
can  be  placed  on  any  of  them:  Bromides,  salicylates,  digitalis,  strophan- 
thus,  iron,  quinine,  belladonna,  iodides  and  injections  of  iodine  into  the 
thyroid  gland. 

Lancereaux  reported  very  brilliant  results  from  the  use  of  quinine 
sulphate  with  and  without  ergot.  Forchheimer  obtained  very  good  results 
from  neutral  salt  of  quinine  hydrobromate  in  gr.  v  doses  three  or  four 
times  daily.  The  drug  should  be  administered  for  a  very  long  time, 
viz.  months.  In  case  of  persistent  tinnitus,  which  the  drug  is  apt  to 
produce,  it  is  advisable  to  discontinue  it  for  a  certain  time  and  then 
resume  it. 

Opotherapy  sometimes  gives  satisfactory  results.  Thyroid  extract, 
thyroidin,  iodothyrin,  antithyroidin,  of  Moebius,  extract  of  parathyroid 
glands,  of  thymus  or  of  suprarenal  capsules  are  administered  internally. 
Milk  of  horses  whose  thyroids  had  been  extirpated  (Lanz),  thyroidectin, 
viz.  desiccated  blood  of  thyroidectomized  sheep,  a  serum  prepared  by 
Rogers  and  Beebe  by  the  use  of  nucleoproteid  and  thyroglobulin  from 
normal  and  pathological  glands,  finally  injection  of  serum  of  thyroidec- 
tomized dogs  (Ballet  and  Enriquez)  have  also  been  advised.  Rogers  and 
Beebe's  investigations  with  their  serum  concerned  a  series  of  480  patients 
in  1909.  The  results  were  as  follows:  15  per  cent,  totally  cured;  10  per 
cent,  cured  from  their  subjective  disturbances,  but  exophthalmos  or 
goiter  remained  unaltered;  50  per  cent,  improved;  17  per  cent,  derived 
no  benefit;  8  per  cent,  died  from  the  progress  of  the  disease.  The  results 
are  better  in  acute  than  in  chronic  cases. 


566  EXOPHTHALMIC    GOITER 

It  is  impossible  as  yet  to  form  a  definite  opinion  as  to  the  therapeutic 
value  of  these  specific  preparations. 

Electricity,  especially  galvanism  with  the  negative  pole  on  the  goiter, 
stabile  galvanization  of  the  sympathetic  may  sometimes  render  some 
service.  X-ray  and  radium  therapy  are  reported  to  be  beneficial.  While 
radiotherapy  may  have  a  favorable  effect  on  the  general  health  and  the 
nervous  manifestations,  it  has  no  effect  on  the  goiter,  exophthalmos 
and  very  slight  effect  on  the  tachycardia.  On  the  other  hand  radio- 
therapy 'may  produce  perithyroid  adhesions  which  may  be  a  disturbing 
element  in  ulterior  operative  procedures.  Moreover,  X-rays  may  produce 
a  thyroid  insufficiency  with  myxcedema  or  else  may  aggravate  the  preex- 
isting manifestations,  as  some  observations  tend  to  show. 

While  the  medical  treatment  rarely  gives  permanent  results,  surgical 
intervention  meets  with  somewhat  greater  success.  Operations  should 
not  be  performed  in  every  case.  In  grave  or  advanced  cases  secondary 
organic  changes  develop  in  the  thyroid,  myocardium,  periocular  tissues, 
so  that  complete  disappearance  of  symptoms  cannot  be  expected.  Pa- 
tients who  reach  a  state  of  cachexia  should  not  be  operated  upon. 

Surgical  intervention  is  indicated  in  cases  which  continue  to  progress 
in  spite  of  the  best  medical  treatment;  in  cases  in  which  there  are  evidences 
of  compression  of  the  trachea,  oesophagus  and  recurrent  laryngeal  nerve; 
in  cases  with  a  very  acute  onset;  in  cases  with  grave  nervous  and  mental 
symptoms;  finally  in  cases  with  a  marked  lymphocytosis  according  to 
Kocher. 

In  the  majority  of  cases  exophthalmic  goiter  is  rebellious  to  medical 
treatment.  However,  an  attempt  with  internal  medications  should  be 
made  almost  in  every  case.  Horsley  recommends  about  six  weeks  of 
such  treatment  and  if  the  results  are  meager,  surgical  intervention  should 
not  be  postponed.  On  the  other  hand  the  best  results  are  obtained  if 
the  patients  are  operated  on  very  early  in  the  course  of  the  disease. 

Thyroidectomy  and  ligation  of  the  thyroid  arteries  are  very  serious 
operations  and  exceptionally  give  favorable  results.  Complete  recoveries 
have  been  reported.  If  under  recoveries  is  understood  a  total  disappear- 
ance of  all  subjective  and  objective  symptoms,  they  are  exceptional.  In 
the  majority  of  cases  considerable  amelioration  is  obtained.  Thyroidec- 
tomy should  always  be  partial  in  view  of  the  great  danger  following 
removal  of  the  parathyroid  glands.  In  ligation  of  thyroid  arteries  there 
is  danger  of  including  the  recurrent  laryngeal  nerve  while  tying  the  in- 
ferior thyroid  artery.  The  danger  is  in  producing  laryngeal  paralysis 
and  inhalation  pneumonia.  Ligation  of  the  superior  thyroid  artery  is, 
according  to  Mayo,  safe. 


MYXCEDEMA 


567 


Removal  of  the  cervical  sympathetic  ganglia  with  their  cord  gives, 
according  to  the  statistics  of  Jonnesco,  the  most  satisfactory  results.  He 
advises  bilateral  extirpation  of  all  the  three  ganglia  with  the  cords.  The 
results  obtained  by  him  have  been  uniformly  perfect.  Chalier  (Lyon 
Chirurgical,  191 1)  in  an  extensive  study  confirms  Jonnesco's  statement, 
viz.  that  operations  on  the  sympa- 
thetic are  in  every  respect  superior 
to  thyroidectomy. 

All  reflex  irritation  of  the  sym- 
pathetic from  some  remote  affec- 
tion, as  fibroid  uterine  tumors, 
nasal  polyps,  etc.,  should  be  re- 
moved. 

If  the  syndrome  of  Grave's  dis- 
ease occurs  during  the  course  of  a 
spinal  or  other  organic  nervous  dis- 
ease, no  operation  should  be  per- 
formed. 


MYXCEDEMA  (CACHEXIA 
STRUMIPRWA) 

This  disease  was  first  described 
by  Gull  in  1873  and  in  1877  by 
Ord,  who  gave  the  above  name. 

Symptoms. — There  are  three 
cardinal  symptoms  that  charac- 
terize the  affection,  viz.  (1)  swell- 
ing of  the  skin,  (2)  atrophy  of  the 
thyroid  gland  and  (3)  mental 
deficiency. 

1.  The  swelling,  which  is  due  to  a  mucous  or  mucoid  infiltration  of 
the  skin,  is  first  noticed  on  the  face.  The  latter  is  large,  round  and  its 
skin  is  of  a  yellowish  tint  (wax-like),  dry  and  without  hair.  On  palpation 
the  skin  feels  hard,  not  depressible  and  thick. 

The  nose  is  enlarged,  the  eyelids  are  swollen  and  droop,  the  lips  are 
thick,  the  forehead  is  wrinkled. 

The  facies  is  without  expression  and  appears  stupid. 

In  other  portions  of  the  body  the  skin  is  equally  thick.  The  trunk 
is  less  affected  than  the  extremities.  The  fingers  and  toes  are  large,  the 
nails  are  thick,  hard  and  brittle. 


100  i 

i®S& 

90  J 

r^mmPQk  •'**  ■Bl 

80  J 

Mfr$*j$k. 

70  1 

JhHb 

60  J 

tBL 

50J 

JUK       1  H 

■He  '           ■895 

Fig.  162.— Myxedematous  Idiocy. 
(Bourneville.) 


568 


MYXCEDEMA 


The  hair  of  the  body  atrophies  and  falls  out,  perspiration  ceases, 
the  skin  is  dry  and  scales  off. 

In  the  subclavicular  region  and  in  the  axilla  pseudo-lipomatous  masses 
are  found. 

The  mucous  membranes  are  equally  swollen,  pale  and  dry.  The 
tongue  is  thickened  and  if  the  pharyngeal  and  laryngeal  mucous  mem- 


Fig.  163. — Infantile  or  Congenital  Myxcedema. 

branes  are  swollen,  dysphagia  and  changes  of  the  voice  are  present     The 
teeth  also  suffer  in  their  nutrition  and  fall  out. 

2.  The  thyroid  gland  is  usually  atrophied.  Very  exceptionally  it  is 
hypertrophied. 

3.  The  mental  condition  is  marked  by  deficient  intellect.  The  patient 
is  somnolent,  apathetic,  his  answers  are  slow,  memory  decidedly  impaired. 
He  is  usually  irritable.     In  some  cases  delirium  and  hallucinations  develop. 


MYXCEDEMA  569 

Other  Symptoms. — The  heart-beat  is  weak,  the  pulse  is  small  and 
irregular.  Hemorrhages,  and  especially  uterine,  are  frequent.  The 
temperature  is  below  normal.  Constipation  is  the  rule.  Patients  often 
complain  of  pain  in  the  extremities  and  neck,  of  deafness,  tinnitus 
aurium,  vertigo  and  headache.  The  reflexes  are  not  altered.  There 
are  no  objective  marked  sensory  disturbances  except  some  diminution  of 
sensations. 

The  patient  is  unable  to  do  any  work.  He  is  slow  in  words,  actions 
and  thinking.     The  gait  is  hesitating.     The  torpitude  is  general. 

Forms. — The  clinical  picture  just  given  presents  a  fully  developed 
type  of  myxcedema  of  adults.  But  there  are  also  incomplete  forms  in 
which  only  some  features  of  the  typical  form  are  present  and  in  which 
thyroid  medication  proves  to  be  beneficial. 

Infantile  or  Congenital  Myxcedema. — What  characterizes  this  form 
is  the  arrest  of  physical  and  intellectual  development. 

Physically  these  patients  are  dwarfs.  The  head  is  narrow  in  front, 
the  anterior  fontanelle  persists.  The  dentition  is  delayed.  The  mouth 
is  open,  the  saliva  dribbles  continuously.  They  begin  to  walk  and  speak 
late.  The  genital  organs  are  not  developed.  The  usual  signs  of  pubes- 
cence are  absent.  These  patients  eat  abundantly  and  are  much  consti- 
pated. Intellectually  they  are  idiots.  When  they  grow  up,  they  have 
the  appearance  as  described  in  the  first  chapter,  except  that  intellectually 
they  show  they  have  never  developed.  Such  children  are  usually  born 
from  tubercular,  alcoholic  or  syphilitic  parents. 

Infantile  myxcedema  may  present  various  degrees.  It  depends  upon 
the  amount  of  thyroid  gland  preserved.  Brissaud's  "infantilism"  be- 
longs to  this  class.  Here  we  find  an  arrest  of  physical  development 
(small  size,  undeveloped  genitalia,  dry  skin  without  hair,  etc.),  but  there 
is  some  degree  of  intellectuality  sufficient  for  certain  occupations.  These 
individuals  are  small  adults.  They  constitute  the  so-called  "partial 
myxcedema"  cases. 

Cases  of  infantilism  described  by  Lorrain  do  not  enter  here.  They 
are  small  individuals,  but  do  not  possess  the  attributes  of  infancy. 

MYXCEDEMA  STRUMIPRIVA  (OPERATIVE  MYXCEDEMA ) 

This  form  may  follow  extirpation  of  the  thyroid  gland  or  of  a  portion 
of  it. 

The  symptoms  begin  to  develop  some  months  after  the  operation, 
although  they  may  appear  sooner.  The  initial  symptoms  are:  a  lassi- 
tude and  weakness  in  the  limbs.     Gradually  the  skin  becomes  infiltrated 


5/0  •  •  MYXCEDEMA 

and  the  hair  begins  to  fall  out.  At  the  same  time  the  characteristic 
mental  hebetude  (see  above)  makes  its  appearance.  The  intensity  of 
the  symptoms  varies^from  case  to  case  and  depends  also  upon  the  age  at 
which  the  operation  is  performed.  The  younger  the  individual,  the 
graver  the  symptoms  are.  A  point  of  great  importance  is  the  fact  that 
partial  thyroidectomy  is  followed  by  far  less  grave  symptoms  than  total 
extirpation  of  the  gland.  Gley  and  Vassale  have  called  attention  to  the 
great  relationship  of  the  parathyroid  glandules  to  physical  and  mental 
conditions.  Preservation  of  these  glandules  is  never  to  be  forgotten  in 
operations  on  the  thyroid  gland. 

Sporadic  Cretinism. — It  presents,  physically  and  mentally,  the  same 
infantile  myxcedematous  type  as  described  above.  The  only  difference 
is  found  in  the  thyroid,  which  is  here  hypertrophied  instead  of  being 
atrophied.  The  hypertrophy  coexists  with  insufficient  function:  instead 
of  glandular  tissue  there  is  proliferation  of  sclerotic  tissue.  Consequently 
from  the  standpoint  of  function  it  is  identical  to  an  atrophic  thyroid. 

This  type  is  particularly  observed  in  certain  localities  of  Switzerland, 
Austria,  Italy  and  France.  Climate  and  geological  conditions,  the  water 
and  the  air,  have  probably  to  do  with  the  disease. 

Course,  Duration,  Prognosis. — In  the  typical  form  the  course  is  slow 
and  progressive.  The  physical  and  intellectual  infirmities  gradually  in- 
creasing lead  to  a  profound  deterioration.  The  skin  gets  very  hard,  the 
functions  of  the  viscera  are  more  and  more  disturbed.  The  temperature 
goes  down,  the  secretions  become  less  and  less.  However,  remissions 
occur,  but  these  periods  of  amelioration  are  only  temporary.  Eventually 
the  mental  hebetude  increases  and  a  pronounced  cachexia  sets  in.  Death 
is  the  ultimate  result.  Death  may  occur  from  some  intercurrent  disease, 
among  which  pulmonary  tuberculosis  is  the  most  frequent.  The  prog- 
nosis is  therefore  unfavorable.  In  the  operative  myxcedema,  as  men- 
tioned above,  the  prognosis  depends  upon  the  integrity  of  the  parathy- 
roid glandules  and  upon  the  amount  of  thyroid  gland  removed  and  finally 
upon  the  age  at  which  the  operation  is  performed.  Grave  immediate 
symptoms  may  also  occur  after  the  operation,  viz.  convulsive  seizures 
and  tetany.  The  latter  particularly  may  persist  for  months  or  cause 
death.  These  phenomena  are  due  to  the  removal  of  the  parathyroid 
glands. 

In  infantile  myxcedema  the  individual  cannot  be  considered  diseased; 
his  health  is  as  a  rule  good.  His  condition  does  not  lead  to  cachexia. 
He  simply  presents  a  morphological  or  functional  anomaly  which  does  not 
compromise  his  existence. 

Diagnosis. — In  the  majority  of  cases  the  characteristic  symptoms  of 


MYXCEDEMA  57 1 

the  skin,  of  the  thyroid  gland,  of  the  intellectuality,  of  the  attitude,  .are 
sufficiently  typical  for  the  recognition  of  myxcedema.  Some  difficulty 
may  be  encountered  in  the  diagnosis  of  the  incomplete  forms.  Infantile 
myxcedema  will  be  recognized  from  the  arrested  development;  there  is 
no  cachexia. 

Etiology. — Heredity  plays  a  certain  role.  Alcoholism,  tuberculosis, 
diabetes  and  syphilis  in  the  parents  are  considered  as  predisposing  causes. 
Pregnancy,  lactation,  menopause,  which  are  accompanied  by  conges- 
tion of  the  thyroid,  perhaps  have  a  predisposing  influence. 

Direct'  causes  are:  infectious  diseases  which  may  lead  to  an  inflamma- 
tion of  the  thyroid  gland  ending  in  its  sclerosis  and  atrophy.  Tumors 
of  the  gland  may  produce  the  same  condition. 

Acquired  myxcedema  may  occur  at  any  age,  but  more  frequently  in 
children  than  in  adults.     Females  are  more  predisposed  than  males. 

In  infantile  myxcedema  the  cause  of  congenital  absence  of  the  thyroid 
gland  is  unknown. 

Pathogenesis. — Pathological  investigations  show  that  in  congenital 
infantile  myxcedema  the  thyroid  gland  is  totally  absent.  In  the  adult 
form  of  myxcedema  there  is  degeneration  of  the  normal  tissue  of  the  thy- 
roid gland,  proliferation  of  connective  tissue.  The  subcutaneous  tissue 
is  infiltrated  with  mucin.     There  is  hyperplasia  of  adipose  tissue. 

The  suppression  of  the  function  of  the  thyroid  gland  is  the  cause  of 
the  disease. 

Normally  the  thyroid  elaborates  a  special  substance  which,  thrown 
into  the  circulation,  destroys  the  usual  toxic  elements  of  the  organism. 
When  the  thyroid  is  diseased,  its  antitoxic  substance  is  either  absent 
or  perverted.  The  result  is  myxcedema.  Internal  administration  of 
thyroid  extract  supplies  the  organism  with  the  necessary  elements  and 
amelioration  of  symptoms  follows.  That  the  parathyroid  glands  partici- 
pate in  this  useful  function  there  can  be  no  doubt  (see  above). 

Treatment. — The  foregoing  remarks  lead  logically  to  the  therapeutic 
indication.  Thyroid  extract  administered  internally  is  almost  the  only 
remedy  for  the  condition.  As  it  may  produce  tachycardia,  cerebral 
irritation,  headache,  insomnia,  dyspncea  and  sometimes  albuminuria, 
its  administration  should  be  watched.  It  is  advisable  therefore  to  com- 
mence with  small  doses — for  an  adult  gr.  j  or  ii  t.  i.  d.  and  for  a  child  a 
fraction  of  a  grain.  The  initial  dose  can  be  gradually  increased.  General 
hygienic  measures  should  not  be  neglected.  A  vegetable  diet  is  preferable 
to  any  other. 

Good  results  have  been  obtained  with  thyroid  not  only  in  the  acquired 
form,  but  also  in  the  congenital  form. 


572 


ACROMEGALY 


ACROMEGALY 

This  disease  was  first  described  by  Pierre  Marie  in  1885. 

Symptoms. — The  essential  and  characteristic  signs  of  the  disease  are 
enlargement  of  the  osseous  and  other  supporting  tissues,  especially  notice- 
able in  the  distal  ends  of  the  extremities  (hands,  feet)  and  the  head. 

The  hands  become  first  affected.  They  are  thick  and  wide.  The 
bones  and  the  soft  parts  are  involved.  The  skin  is  hard  and  free  of  oedema. 
The  thenar  and  hypothenar  eminences  are  very  large.  The  fingers  are 
enormous,  but  not  deformed  and  their  function  is  preserved.  The  fingers 
may  be  only  wide  (transverse  type)  but  also  long  and  wide  (longitudinal 


Fig.  164. — Acromegaly.     (Marie.) 


type).  The  size  of  the  hand  is  out  of  proportion  with  the  rest  of  the  upper 
limb  which  is  only  slightly  enlarged.  The  feet  are  equally  enlarged.  The 
toes  and  the  soft  tissues  are  thick  and  wide.  Here  again  the  contrast 
between  the  size  of  the  feet  and  the  legs  is  striking;  the  latter  are  only 
slightly  involved.  On  the  head  the  occipital  protuberance  is  prominent. 
The  cranial  bones  are  irregularly  thickened.  It  is  particularly  the  face 
that  shows  hypertrophy.  It  is  elongated,  the  forehead  is  low,  but  the 
orbital  and  zygomatic  arches  are  very  prominent,  the  eyelids  are  thick- 
ened.    The  nose  is  enormous.     The  lower  jaw  is  very  much  hypertro- 


ACROMEGALY  573 

phied,  the  chin  is  projected  (prognathism)  and  large.  The  lips  are  heavy 
and  the  lower  one  is  everted.  The  tongue  is  thick  (macroglossia)  and 
broad.  The  palate,  tonsils,  uvula  and  larynx  are  all  hypertrophied.  The 
vocal  cords  are  thick.  The  mucous  membranes  are  hypertrophied  so 
that  difficulty  of  deglutition  may  occur  and  the  voice  is  deep.  The  skin 
of  the  face  is  dry  and  brownish.     The  hair  of  the  head  is  thickened. 

The  vertebral  column  presents  a  cervico-dorsal  kyphosis. 

The  sternum  is  thickened  and  projected;  the  clavicle  and  the  ribs  are 
large;  the  costal  cartilages  are  ossified. 

Besides  the  deformities  there  are  other  constant  symptoms:  Headache 
and  Amenorrhea.     They  often  appear  at  the  onset  of  the  disease. 

Other  Symptoms. — Not  infrequently  ocular  disturbances  occur,  viz. 
scotomata,  diplopia,  strabismus,  diminution  of  visual  acuity;  also  optic 
neuritis  and  optic  atrophy.  Blindness  and  hemianopsia  with  Wernicke's 
pupillary  reaction  are  occasionally  observed.  In  a  large  number  of  cases 
simple  amaurosis  with  pupillary  immobility  is  observed. 

The  viscera  sometimes  participate  in  the  hypertrophy.  Cardiac 
hypertrophy  is  particularly  troublesome.  Palpitation,  arhythmia  and 
dyspnoea,  also  attacks  of  syncope  are  quite  frequent. 

The  abdomen  is  large,  the  mammary  glands  and  the  uterus  are  atro- 
phied. The  male  genitalia  are  enlarged,  but  impotence  is  present.  In 
the  female  the  clitoris  may  undergo  hypertrophy. 

Sterility  is  frequent. 

Polydypsia,  polyuria  glycosuria  are  quite  frequent. 

Sensations  are  normal,  but  pain  in  the  extremities  and  spine  is  fre- 
quent. It  is  increased  by  fatigue,  pressure  and  exposure  to  cold.  The 
pain  may  be  of  neuralgic  or  rheumatoid  type. 

The  reflexes  may  be  normal,  increased  or  diminished. 

There  is  a  general  sense  of  continuous  fatigue,  although  the  movements 
of  the  limbs  are  not  involved. 

The  patient  is  as  a  rule  apathetic  and  of  slow  mentality. 

Focal  epilepsy  has  been  observed  in  exceptional  cases. 

Course,  Duration,  Prognosis. — The  course  is  usually  slow  but  pro- 
gressive. The  disease  may  follow  an  acute  or  chronic  course.  The  latter 
is  the  most  frequent.  Amelioration  and  temporary  arrest  occur.  It 
usually  lasts  twenty  or  thirty  years.  Cachexia  and  death  are  the  habitual 
termination,  unless  the  patient  dies  from  some  intercurrent  disease. 
Men  are  more  frequently  affected  than  women. 

Diagnosis. — The  differentiation  with  Myxoedema  is  comparatively 
easy.  In  the  latter  disease  the  enlargement  of  the  extremities  is  due  to 
a  mucoid  infiltration  of  the  skin.     The  round,  puffy  face  of  a  myxcedema- 


574  ACROMEGALY 

tous  is  essentially  different  from  the  oblong  face  and  prognathism  of  an 
acromegalic. 

In  Elephantiasis  only  the  skin  and  the  cellular  tissue  are  involved. 
It  affects  only  one  side  and  an  entire  limb. 

Hypertrophic  Pulmonary  Osteo -arthropathy  (Marie)  is  characterized 
by  paw-like  hands,  the  ends  of  the  fingers  are  enlarged  and  the  nails  are 
thick  and  brittle.  The  lower  jaw  is  not  involved.  The  disease  is  usually 
associated  with  cardiac  or  pulmonary  diseases. 

In  Osteitis  deformans  (Paget)  there  is  a  marked  deformity  of  the  legs, 
but  the  hands  and  feet  are  intact.  The  cranium  is  deformed,  while  in 
acromegaly  the  face  is  involved. 

Etiology. — Heredity  plays  a  small  part.  Diseases  of  the  nervous 
system  (chorea,  hysteria,  tabes),  mental  diseases,  infectious  diseases, 
alcoholism,  syphilis,  gout  have  all  been  considered  as  predisposing  factors. 
As  exciting  causes  can  be  mentioned  trauma  and  violent  emotions.  The 
disease  occurs  mostly  at  the  age  of  between  twenty  and  thirty-five. 
Females  are  somewhat  more  frequently  affected  than  males. 

Pathogenesis. — Pathological  investigations  show  that  in  a  large 
majority  of  cases  the  pituitary  body  was  found  diseased  (degeneration, 
tumor,  hypertrophy).  In  a  number  of  cases  the  thyroid  gland,  the 
thymus,  suprarenals  and  pancreas  were  altered.  Although  in  some 
observations  the  pituitary  gland  was  found  intact,  the  consensus  of 
opinion  at  present  is  that  a  disturbance  in  function  of  this  glandular 
body  is  the  only  cause  of  acromegaly  (hyperpituitarism).  There  are 
clinical  and  experimental  reasons  to  believe  in  physiological  interrelation 
between  all  those  glands. 

The  nutrition  of  the  body  and  cartilaginous  tissues  is  supposed  to 
be  controlled  by  the  pituitary  body.  Therefore  any  disturbance  in  its 
normal  function  is  followed  by  the  manifestations  characteristic  of 
acromegaly. 

Treatment. — The  foregoing  remarks  lead  to  therapeutic  indications. 
The  pituitary  body,  thyroid  and  thymus  glands  have  been  administered  in 
acromegaly.  Some  favorable  results  have  been  reported,  particularly  from 
the  use  of  the  pituitary  gland.  Iodides,  mercury,  iron,  ergot  and  arsenic 
may  be  tried.  Other  manifestations  of  the  disease  are  treated  sympto- 
matically.  General  hygienic  measures  should  not  be  neglected.  Opera- 
tive procedures  for  the  purpose  of  removing  the  hypophysis  date  from 
1907.  Schlosser  was  the  first  to  extirpate  a  tumor  in  a  man.  Surgeons 
have  since  attempted  operations  through  different  routes.  The  latter 
are:  (1)  nasal  route  combined  with  resection  of  the  upper  maxilla  and 
orbit;  (2)  buccal  route  with  resection  of  hard  palate;  (3)  transverse  supra- 


GIGANTISM 


575 


hyoid  pharyngotomy;  (4)   temporal  intra-cranial  route;  (5)  trans-frontal 
method  (Frazier),  viz.  through  anterior  cranial  fossa. 


GIGANTISM 

A  condition  allied  to  acromegaly  is  gigantism.  Under  this  name 
should  be  understood  a  condition  which  is  characterized  not  only  by  a 
size  of  the  body  above  the  normal,  but  also 
by  physical  and  mental  anomalies. 

Symptoms. — The  most  important  feature 
is  the  excessive  size  of  the  body.  The 
height  may  reach  nine  or  ten  feet.  The  un- 
usual growth  of  the  body  commences  at 
puberty  and  in  exceptional  cases  in  childhood 
and  continues  above  the  average  age. 

In  spite  of  their  enormous  size  the  giants 
complain  of  muscular  weakness. 

As  a  sign  of  abnormality  should  be  also 
mentioned  atrophy  of  genital  organs,  the  con- 
sequence of  which  are  impotence,  sterility, 
amenorrhea. 

The  mentality  is  also  below  normal.  Such 
patients  are  apathetic,  childish,  without  initia- 
tive and  with  a  poor  memory. 

As  additional  manifestations  they  infre- 
quently present  continuous  headache,  visual 
disturbances,  polyuria  and  especially  gly- 
cosuria. 

Gigantism  and  acromegaly  is  one  affection. 
If  the  progressive  increase  in  size  makes  its 
appearance  in  childhood  or  youth,  the  result 
will  be  gigantism.  If  the  size  continues  to 
increase  beyond  the  age  at  which  growth 
ceases  normally,  the  result  will  be  associated  with  acromegaly  (see  this 
chapter).  The  individual  is  therefore  an  acromegalic  giant.  Acro- 
megaly is  consequently  the  gigantism  of  the  period  of  full  development. 
Gigantism  is  acromegaly  of  the  developmental  period.  The  association 
of  gigantism  and  acromegaly  may  occur  not  only  in  two  successive  periods, 
which  is  the  rule,  but  also  simultaneously  long  before  the  epiphyseal 
cartilages  become  ossified,  at  a  period  very  near  birth  and  even  before 
birth.     In  the  latter  case  the  hypophyseal  hypertrophy,  which  is  very 


Fig. 


165. — Acromegalic  Giant. 
(Brissaud  and  Meige.) 


576  ACHONDROPLASIA 

probably  the  common  cause  of  both  gigantism  and  acromegaly,  may 
sometimes  be  congenital.  Gigantism  may  be  associated  with  infantilism. 
In  such  individuals  the  attributes  of  an  infant  are  present,  as,  for  example, 
absence  of  ossification  of  the  cartilages  between  the  diaphysis  and  epiphysis. 
Moreover,  there  are  also  atrophy  of  the  genitalia,  undeveloped  muscu- 
lature, tender  skin,  absence  of  hair  of  the  pubis  and  axilla.  The  mentality 
is  that  of  a  young  child.     An  infantile  giant  may  also  become  acromegalic. 

Course,  Duration,  Prognosis. — In  the  majority  of  cases  the  course 
is  slow,  although  acute  cases  have  been  reported  which  ended  fatally  at 
an  early  age.  Ameliorations  have  been  observed.  The  condition  is 
essentially  progressive.  The  patients  usually  die  from  some  intercurrent 
disease,  particularly  pulmonary  tuberculosis. 

Pathogenesis  and  Etiology. — Pathological  investigations  show  in  the 
majority  of  cases  a  diseased  condition  of  the  pituitary  body,  either  hyper- 
trophy or  tumor.  In  some  cases  hypertrophy  of  the  thyroid  gland  was 
found. 

The  prevalent  opinion  is  that  gigantism  is  due  to  the  persistence  of 
the  epiphyseal  cartilages  beyond  the  normal  age  and  continuation  of  the 
growth  of  the  skeleton.  Ossification  of  cartilages  is  controlled  mainly 
by  the  pituitary  bcdy,  but  also  by  the  thyroid,  testicle  and  other  glands 
with  an  internal  secretion.  A  morbid  condition  of  the  latter  will  interfere 
with  the  process  of  ossification.  Speaking  generally  gigantism  is  by  it- 
self an  indication  of  a  degenerative  make-up. 

Gigantism  may  follow  infectious  diseases,  intoxications.  It  affects 
males  more  frequently  than  females. 

Treatment. — Pituitary  or  thyroid  gland  may  be  tried,  although  no 
genuine  satisfactory  results  have  been  reported. 

ACHONDROPLASIA 

It  is  a  congenital  malformation  characterized  by  a  smallness  of  sta- 
ture and  due  to  a  deficient  ossification  of  the  cartilages  of  the  long  bones. 

The  first  good  description  was  given  by  P.  Marie  in  1900,  although 
the  condition  had  been  known  long  before. 

Symptoms.,- — Soon  after  birth  the  unusually  small  size  and  short 
limbs  are  noticed.  The  growth  is  retarded  and  the  individual  reaches 
adult  life,  but  is  a  dwarf. 

The  following  three  peculiarities  characterize  achondroplasia:  dwarf  - 
ishness,  micromyelia  and  macrocephalia. 

The  trunk  and  head  are  of  normal  size,  but  the  limbs  and  particularly 
the  lower  are  unusually  short  (micromyelia) .     The  contrast  is  striking  and 


ADIPOSIS    DOLOROSA 


577 


typical.  The  proximal  ends  of  the  limbs  (arms  and  thighs)  are  more 
affected  than  the  distal  ends.  Normally  when  the  individual  is  in  a 
standing  position,  the  medius  reaches  the  lower  third  of  the  thigh.  In 
achondroplasia  the  same  finger  hardly  reaches  the  trochanter. 

Marie  called  attention  to  a  special  position  of  the  fingers.     When 
an  attempt  is  made  to  bring  them  together, 
only  the  first  phalanges  are  approached,  the 
last  two  are  separated. 

The  head  may  be  of  normal  size,  but 
not  infrequently  is  enlarged  (macrocephalia). 
Sometimes  it  resembles  a  hydrocephalic 
head. 

Other  Symptoms. — The  scapulae  are 
short.  The  pelvis  is  small.  The  muscula- 
ture on  the  contrary  is  well  developed.  The 
genitalia  are  normal  in  size  and  function. 
The  mentality  in  the  majority  of  cases  is 
normal.  In  Marie's  cases,  however,  it  was 
deficient. 

Pathogenesis  and  Etiology. — Various 
views  have  been  advanced  as  to  the  nature 
of  achondroplasia. 

Some  believe  in  a  hereditary  degenerative 
state  of  the  cartilages.  According  to  others 
a  toxic  material  influences  in  some  manner 
the  nervous  system,  which  in  its  turn  pro- 
duces trophic  disturbances  of  the  cartilages. 
Perhaps  the  ductless  glands  are  the  source 
of  the  toxic  material. 

Treatment. — Not  much  reliance  can  be 
placed  upon  the  administration  of  thyroid 
extract,  which  has  been  advised. 


Fig. 


166. — Achondroplasia. 
(Apert.) 


ADIPOSIS  DOLOROSA 

It  is  characterized  by  deposits  of  fat  in  various  portions  of  the  body. 
These  deposits  are  painful  upon  pressure. 

;  The  disease  has  been  observed  and  described  by  clinicians  under 
various  names,  but  Dercum  was  the  first  to  give  this  clinical  entity  the 
name  of  adiposis  dolorosa. 

Symptoms. — Four  main  symptoms  characterize  the  disease,  viz.  fat 

37 


578  ADIPOSIS    DOLOROSA 

formation,  pain,  asthenia  and  psychic  symptoms.  The  striking  feature 
is  the  nodes  of  fat  distributed  all  over  the  body.  The  deposition  of  fat 
may  be  not  only  nodular,  but  also  circumscribed  diffuse  or  generalized 
diffuse.  The  nodular  is  the  most  common  form.  The  extremities  are 
particularly  involved.  At  first  the  bunches  of  fat  are  noticed  in  the  lower 
and  upper  extremities;  the  trunk  is  affected  later,  but  the  face,  hands  and 
feet  have  been  spared  in  all  observed  cases.  When  these  nodes  are 
compressed,  the  patient  suffers  considerable  pain.  In  the  nodular  form 
the  size  of  the  nodules  may  be  as  small  as  a  pea  but  more  frequently 
they  are  of  the  size  of  an  orange.  They  are  usually  mobile,  but  they 
may  be  adherent  to  the  skin.  In  the  circumscribed  diffuse  form  the  mass 
may  involve  a  segment  of  a  limb  or  an  entire  limb.  The  mass  may  be 
smooth  or  lobulated. 

The  generalized  diffuse  form  usually  begins  on  the  abdomen  and  then 
spreads  gradually  to  the  trunk  and  extremities.  The  hands  and  feet 
which  usually  remain  unaffected  in  the  two  other  forms,  here  are  not  in- 
frequently involved,  but  the  fatty  deposits  are  seen  only  on  the  palmar 
surface  of  the  hand  (thenar  and  hypothenar  regions)  and  on  the  soles 
of  the  feet. 

The  skin  over  the  involved  area  is  usually  white  and  soft.  Pigmenta- 
tion of  the  skin  with  atrophy  and  diminished  sweating  have  been  observed. 

Other  Symptoms. — The  patient  often  complains  of  spontaneous  dull 
aching,  also  of  numbness,  coldness  or  else  burning.  Pain  is  never  absent. 
It  may  be  spontaneous  or  elicited  by  pressure.  In  well-advanced  cases 
it  may  be  unusually  severe.  It  does  not  follow  the  course  of  nerve  trunks. 
Besides  being  continuous,  the  pain  may  present  paroxysms  of  unusual 
severity.  At  each  of  such  paroxysms  the  size  of  the  fatty  mass  increases, 
but  recedes  at  the  disappearance  of  pain.  There  is  usually  a  marked 
diminution  of  objective  sensibility  or  complete  anaesthesia  in  the  affected 
areas.  Muscular  weakness  is  a  frequent  symptom.  Sometimes  it  is 
so  marked  that  the  patient  is  compelled  to  remain  in  bed.  Headache, 
hemorrhages  in  the  nasal  cavities  occasionally  occur.  One  of  my  patients 
presented  retinal  hemorrhages  appearing  without  a  strain,  effort  or  any 
traceable  cause.  Contraction  of  the  visual  fields,  scotomata,  amaurosis 
have  been  observed.  Psychic  symptoms  are  quite  frequent.  Change  of 
disposition,  marked  irritability  are  common.  In  some  cases  there  is  also 
mental  exhaustion.  Delusions  of  persecution,  dementia  have  also  been 
observed. 

Vaso-motor  disturbances  are  not  rare.  Cyanosis  of  the  limbs, 
oedema,  ecchymosis  upon  the  least  trauma  or  without  any  apparent  cause 
have  been  also  observed. 


SCLERODERMA  579 

Among  the  rare  manifestations  arthropathies  have  been  recorded. 

Course,  Duration,  Prognosis. — The  disease  is  essentially  progressive. 
Its  duration  is  indefinite.  Death  usually  occurs  from  some  intercurrent 
disease,  pulmonary  especially. 

Diagnosis. — The  disease  cannot  be  confounded  with  myxcedema, 
first  because  of  its  localization  and  next  because  of  the  pain  upon  compres- 
sion of  the  enlarged  areas  of  the  skin. 

Etiology. — A  neuropathic  tendency  was  traced  in  almost  every  case 
so  far  reported.  Alcohol  and  syphilis  may  have  some  etiological  impor- 
tance. Disturbances  of  sexual  functions  (menopause,  menorrhagia,  abor- 
tion) have  been  reported  as  immediately  preceding  the  onset.  Trauma, 
emotion,  exposure  to  cold  have  been  traced  in  some  cases  as  immediately 
preceding  the  onset.     Women  are  more  frequently  affected  than  men. 

Pathogenesis. — Autopsies  have  so  far  failed  to  reveal  the  true  nature 
of  the  disease.  Cases  have  been  reported  with  morbid  changes  in  the 
thyroid  gland  and  pituitary  body.  In  one  of  Dercum's  patients  there 
was  an  interstitial  neuritis  with  a  moderate  sclerosis  of  Goll's  columns. 
The  general  opinion  is  that  the  affection  is  due  to  a  connective  tissue 
dystrophy  (fatty  changes)  with  an  involvement  of  the  nerve-fibers 
(neuritis) . 

Treatment. — The  foregoing  remarks  suggest  a  trial  of  thyroid  therapy. 
In  my  case  mentioned  above  I  obtained  amelioration  of  the  parassthetic 
disturbances  from  internal  administration  of  extract  of  parathyroid  glan- 
dules. Avoidance  of  fatigue  and  general  hygienic  measures  are  not  to  be 
neglected.  Pain  may  be  relieved  by  coal-tar  products.  Prolonged  rest 
in  bed  may  be  beneficial. 

SCLERODERMA 

It  is  chacarterized  by  an  induration  of  the  skin  and  its  adherence  to 
the  subcutaneous  tissue. 

Symptoms. — Before  the  formation  of  the  characteristic  skin  the  patient 
complains  of  distressing  subjective  sensations,  as  pain,  itching,  tingling, 
etc.  Soon  cedematous  spots  appear  on  the  skin;  they  may  remain  cir- 
cumscribed (morphea)  or  merge  one  into  another  and  become  diffuse. 
Gradually  atrophy  of  the  skin  develops,  so  that  in  an  advanced  stage  the 
skin  is  thin,  tense,  hard  and  glossy.  Pigmentation,  vitiligo  are  noticeable. 
The  skin  cannot  be  wrinkled. 

In  still  more  advanced  period  the  atrophy  progresses  and  involves  the 
underlying  tissues.  The  subcutaneous,  muscular  and  osseous  tissues 
become  invaded. 


580  SCLERODERMA 

The  disease  may  be  localized  (morphea)  or  diffuse,  bilateral  or  uni- 
lateral. The  most  frequent  seat  of  the  affection  is  on  the  face,  neck, 
upper  part  of  the  thorax  and  upper  extremities,  especially  the  hands. 
When  the  face  is  involved,  the  features  are  drawn,  immobile,  mask-like, 
the  lips  are  thin  and  the  display  of  the  muscles  in  emotions  is  of  course 
disturbed.     Mastication  and  speech  are  difficult. 

When  the  hands  are  affected,  the  fingers  are  rigid,  thin  and  retracted 
(sclerodactyly) .  Constriction  and  subsequently  spontaneous  amputation 
of  phalanges  may  occur.  Raynaud's  disease  may  sometimes  complicate 
sclerodactyly. 

When  the  neck  and  thorax  are  affected,  the  respiration  may  be  inter- 
fered with.  Sclerodema  has  been  observed  in  association  with  trophic 
disturbances  in  other  parts  of  the  body,  viz.  atrophy  of  one-half  of  the 
body,  facial  hemiatrophy. 

Course,  Duration,  Prognosis.- — In  the  large  majority  of  cases  the 
disease  is  essentially  progressive.  Cachexia  and  death  are  the  ultimate 
terminations.  Frequently  the  patient  dies  from  some  intercurrent 
disease. 

Scleroderma  is  sometimes  seen  complicated  by  facial  hemiatrophy  and 
erythromelalgia. 

Raymond  and  Guillain  have  observed  a  case  of  generalized  sclero- 
derma with  bilateral  ocular  palsies  and  nystagmus.  The  latter  symptoms 
were  due  to  sclerosis  of  the  ocular  muscles,  but  not  to  a  genuine  paralysis. 

Etiology. — Scleroderma  has  been  observed  in  the  course  of  lepra, 
syringomyelia,  paresis  and  tabes.  A  neuropathic  and  arthritic  predis- 
position play  some  part.  Traumatism,  emotion,  exposure  to  cold, 
pregnancy  have  been  considered  as  etiological  factors.  The  disease  may 
occur  at  any  age,  even  in  infants,  but  particularly  between  twenty  and 
forty  years  of  age.     Women  are  more  frequently  affected  than  men. 

Pathogenesis. — Pathologically  the  affection  is  characterized  by  a 
sclerosis  affecting  the  skin  and  subcutaneous  tissue.  Atrophy  and 
retraction  follow.  The  bloodvessels  are  also  involved:  a  peri-  and  end- 
arterities  are  always  present. 

These  facts,  however,  do  not  explain  the  nature  of  the  disease.  Some 
authors  believe  that  the  vascular  lesion,  which  is  so  constant,  is  the  only 
cause  of  the  disease.  Others  consider  it  as  a  trophoneurosis  or  angio- 
trophoneurosis.  Others  believe  in  a  sympathetic  origin  and  still  others 
in  infection.  Finally  a  disturbance  of  the  function  of  the  thyroid  gland 
may  have  something  to  do  with  the  scleroderma. 

Treatment. — Thyroid  extract,  iodides,  local  massage,  electricity,  also 
hypodermic  use  of  thiosinamin  (15  per  cent,  of  alcoholic  solution)  every 


FACIAL    HEMIATROPHY 


581 


other   day  are   all  the  remedies   advised, 
placed  on  any  of  them. 


Not  much  reliance   can  be 


PROGRESSIVE  FACIAL  HEMIATROPHY 

Symptoms. — In  the  majority  of  cases  one  or  two  brownish  spots 
appear  first  on  one  side  of  the  face.  The  underlying  skin  begins  to  appear 
glossy   and  to   undergo   atrophic   changes.     The   subcutaneous   cellular 


Fig.  167. — Facial  Hemiatrophy. 

tissue  follows.  A  depression  develops  because  of  the  disappearance  of 
fat. 

The  skin  of  the  nose,  orbit,  cheek  and  lips  becomes  thin  and  wrinkled. 

Other  trophic  disturbances  occur.  The  teeth,  the  eyebrows  fall  out, 
the  secretion^of  the  sebaceous  and  sweat  glands  is  diminished. 


582  FACIAL    HEMIATROPHY 

The  face  is  asymmetrical  and  drawn  to  the  normal  side.  The  contrast 
between  both  sides  is  striking.  The  function  of  the  muscles  of  the  affected 
side  is  as  a  rule  but  slightly  disturbed,  as  there  is  no  paralysis.  Their 
electrical  reactions  show  no  reactions  of  degeneration.  The  faradic  con- 
tractibility  is  increased.  However,  an  associated  hemiatrophy  of  the 
masticators,  of  the  tongue  and  of  the  palate  which  is  occasionally  observed, 
may  interfere  with  mastication.  The  bones  of  the  face  participate  in 
the  atrophy  in  advanced  cases.  The  frontal,  molar  and  the  maxillary 
bones,  the  nasal  cartilages  all  atrophy  and  shrink. 

Sensations  are  generally  not  disturbed,  although  in  my  case  (N.  Y. 
Med.  Jour.,  January,  1907)  they  were  markedly  altered.  The  patient 
frequently  complains  of  paresthesias  (chilliness,  numbness,  etc.).  Pain 
of  a  neuralgic  character  is  sometimes  present.  In  my  case  the  disease 
began  with  severe  pain  around  the  right  infraorbital  foramen,  which  lasted 
two  days;  a  few  days  later  the  hemiatrophy  commenced. 

Course,  Duration,  Prognosis.- — The  hemiatrophy  is  not  always  pre- 
ceded by  appearance  of  brownish  spots.  In  my  case  a  trigeminal  neural- 
gia of  two  days'  duration  was  almost  immediately  followed  by  the  begin- 
ning of  the  atrophy.  The  disease  is  progressive.  Sometimes  the  other 
side  of  the  face  becomes  involved.  Sometimes  only  a  few  symptoms  are 
present  (incomplete  form).  Sometimes  other  parts  of  the  body  may  be 
implicated.     The  prognosis  as  to  life  is  good. 

The  disease  has  been  observed  in  association  with  tabes,  syringomyelia, 
multiple  sclerosis,  epilepsy,  chorea,  facial  tic.  Dilatation,  contraction 
and  immobility  of  the  pupil,  also  congenital  palsy  of  ocular  muscles, 
have  also  been  observed. 

Etiology  and  Pathogenesis. — Facial  neuralgia,  trauma,  infectious 
diseases  or  localized  infectious  processes,  organic  nervous  diseases  (syringo- 
myelia) are  all  possible  causes. 

There  are  at  present  two  views  concerning  the  nature  of  the  affec- 
tion. According  to  some  observers,  a  primary  atrophy  of  the  subcuta- 
neous cellular  tissue  is  the  essential  feature.  Others  believe  that  hemi- 
atrophy is  of  a  nervous  origin  (trophoneurosis)  and  any  of  the  following 
nerves  is  supposed  to  be  the  immediate  cause:  the  sympathetic,  trigem- 
inus or  facial.  That  the  Gasserian  ganglion  and  a  cerebral  lesion  may 
also  produce  the  disease,  there  are  pathological  proofs. 

Treatment. — Local  massage  and  galvanism  may  be  tried.  Gersuny's 
method  of  subcutaneous  injections  of  paraffin  should  be  undertaken  for 
remedying  the  asymmetry  of  the  face.  The  latter,  however,  is  only  an 
esthetic  measure. 


ACROPARESTHESIA  583 

FACIAL  HEMIHYPERTROPHY 

It  is  a  congenital  condition.  It  is  due  to  an  anomaly  in  the  develop- 
ment of  the  face.  It  is  sometimes  associated  with  a  congenital  hernihy- 
pertrophy  of  an  entire  half  of  the  body.  It  consists  of  an  increased  de- 
velopment of  the  bony  as  well  as  of  all  the  covering  tissues  of  one  side 
of  the  face.  The  skin  is  thick  and  coarse.  Not  only  the  superficial  bones 
are  involved,  but  also  the  palate,  basilar  process  and  sphenoid  bone. 

ACROPARESTHESIA 

Symptoms. — The  disease  is  characterized  chiefly  by  paresthesias  in 
the  distal  ends  of  the  extremities,  viz.  fingers  and  occasionally  in  the  toes. 
The  patient  complains  of  a  burning,  tingling,  pins  and  needles'  sensation, 
of  pain  in  the  tips  of  the  fingers  or  toes.  The  hands  are  most  frequently 
involved.  The  disturbance  is  continuous,  but  in  some  cases  it  becomes 
aggravated  at  night  or  early  in  the  morning.  It  is  also  worse  when  the 
fingers  come  in  contact  with  cold  objects,  when  exposed  to  cold  or  when 
placed  in  cold  water.  The  affection  is  usually  bilateral.  There  are  no 
objective  disturbances.  The  color  of  the  skin  is  normal.  The  power  of 
the  hands  as  well  as  their  function  is  also  intact. 

Course,  Duration,  Prognosis. — The  onset  is  slow  in  the  majority  of 
cases.  The  disease  lasts  many  years.  Recovery  is  possible,  but  not  fre- 
quent. The  prognosis  is  good  as  to  life.  The  disease  incapacitates  to  a 
certain  extent  for  physical  work,  as  the  latter  increases  the  parsesthesias. 

Etiology. — Undue  exertion,  exposure  to  cold  are  the  exciting  causes. 
Anaemia  and  pregnancy  are  predisposing  factors.  Women  are  more 
frequently  affected  than  men.  Washerwomen  are  particularly  apt  to 
develop  the  disease.     The  affection  rarely  occurs  before  the  age  of  thirty. 

Pathogenesis. — The  disease  is  considered  as  a  vaso  -motor  neurosis. 
Through  some  influence  the  arteries  are  in  a  state  of  spasm  and  thus  the 
sensory  nerve-endings  not  sufficiently  nourished  are  being  irritated.  In 
advanced  cases  it  is  not  impossible  that  a  neuritis  develops. 

Treatment. — Avoidance  of  fatigue  and  of  exposure  to  cold  is  the  first 
indication.  Arsenic,  phosphorus,  iron  and  quinine  have  been  recom- 
mended. I  have  obtained  favorable  results  from  nitroglycerine.  Very 
recently  I  studied  a  series  of  cases  in  which  I  have  systematically  applied 
Bier's  method  of  induced  hyperemia.  The  results  were  very  encouraging. 
A  bandage  was  applied  for  an  hour  twice  daily  at  the  middle  of  the  fore- 
arm (see,  for  details,  Therapeutic  Gazette,  1908).  Galvanism  and  static 
electricity  have  also  been  advised. 


584  ANGIONEUROTIC    (EDEMA 

ANGIONEUROTIC  (EDEMA  (QUINCKE) 

Symptoms. — The  disease  is  characterized  chiefly  by  paroxysmal 
swellings  of  the  skin  which  are  circumscribed  and  not  inflammatory  in 
nature,  The  mucous  membranes  are  equally  apt  to  be  involved.  The 
favorite  seat  is  on  the  face  and  lips,  but  may  also  occur  on  the  scalp, 
forehead,  palate,  pharynx,  larynx  and  viscera  (stomach  and  intestines) , 
in  rare  cases  in  the  brain  (Osier) .  The  articulations  may  also  be  involved 
by  a  sudden  effusion.  In  one  case,  a  girl  of  eighteen,  the  swellings 
appeared  in  the  upper  eyelids. 

The  circumscribed  swelling  resembles  urticaria;  it  is  round,  of  about 
2  cm.  in  diameter;  it  is  usually  white,  but  it  may  be  also  somewhat  reddish. 
It  does  not  pit  on  pressure. 

The  swellings  may  last  a  few  hours  or  days.  They  frequently  dis- 
appear from  one  part  of  the  body  to  appear  in  another.  The  patient 
does  not  suffer  any  special  discomfort  except  some  tension  and  stiffness, 
but  no  pain.  When  the  mucous  membranes  are  involved,  the  disturbance 
may  interefere  with  the  function  of  the  pharynx,  larynx,  etc.  Difficulty 
of  swallowing  and  of  breathing  will  ensue,  and  in  some  cases  trache- 
otomy may  become  urgent.  In  exceptional  cases  albuminuria  and  hemo- 
globinuria have  been  observed.  When  the  gastric  and  intestinal  mucous 
membranes  are  affected,  vomiting  and  diarrhoea  will  follow. 

Course,  Duration,  Prognosis.— The  disease  may  disappear  sponta- 
neously. In  some  cases  it  may  last  indefinitely.  Recurrences  are  frequent. 
The  prognosis,  while  not  unfavorable,  is,  however,  uncertain,  as  the  disease 
is  a  very  stubborn  one.     It  is  serious  when  the  larynx  is  involved. 

Diagnosis. — The  paroxysmal  character  of  the  swellings,  their  circum- 
scribed appearance,  the  absence  of  pain  and  the  fact  that  they  do  not  pit 
on  pressure  are  all  sufficiently  typical  signs  for  the  diagnosis  and  cannot 
be  confounded  with  other  affections. 

Etiology. — Heredity  and  neuropathic  tendency  play  a  prominent  role. 
It  is  sometimes  observed  in  several  members  of  the  same  family.  It  is 
not  infrequently  associated  with  hysteria,  epilepsy,  exophthalmic  goiter. 

Physical  and  mental  exhaustion  are  also  predisposing  factors.  As 
exciting  causes  may  be  mentioned:  cold,  trauma,  emotion,  also  toxic 
substances,  such  as  alcohol  and  tobacco.  Malarial  poison  may  also 
produce  the  disease. 

Males  are  more  frequently  affected  than  females.  It  usually  occurs 
in  young  age. 

Pathogenesis. — The  disease  is  probably  a  vaso-motor  neurosis,  result- 
ing either  in  a  paralysis  of  the  vaso-constrictors  or  stimulation  of  the 


ERYTHROMELALGIA  585 

vaso-dilators.  The  consequence  is  a  serious  exudation.  The  latter  is  in 
the  subdermal  tissue. 

Treatment. — In  view  of  the  neuropathic  taint  present  in  the  majority 
of  patients,  a  well-regulated  life  with  plenty  of  rest  and  moderate  exercises, 
also  nutritious  food,  hydrotherapy,  massage  and  other  hygienic  measures 
are  the  first  indication  in  the  treatment. 

As  drugs  the  following  can  be  recommended:  strychnia,  atropin, 
quinine,  nitroglycerine. 

The  condition  of  the  pharynx  and  larynx  should  be  watched,  as 
operative  measures  may  be  necessary. 

Alcohol,  tobacco,  or  other  stimulants  are  forbidden. 

HEREDITARY  (EDEMA  OF  THE  LEGS  (MILROY'S  DISEASE) 

It  was  first  described  in  1892  by  Milroy. 

Symptoms. — (Edema  may  appear  soon  after  birth  or  develop  at  the 
age  of  puberty.  It  is  ordinarily  confined  to  the  legs  up  to  the  knees,  but 
it  may  be  confined  only  to  the  ankles  or  even  reach  the  thighs.  The  skin 
of  the  swollen  parts  is  usually  pale.  Acute  attacks  have  been  described. 
In  such  cases  the  swelling  increases,  the  skin  becomes  red  and  some 
constitutional  symptoms  are  present,  viz.  chills,  fever,  vomiting  and  pain 
in  the  abdomen.  The  chief  features  of  this  disease  are:  (1)  absence  of 
all  local  or  general  causes  of  oedema,  (2)  duration — mostly  from  birth, 
(3)  the  familial  character  and  the  presence  of  a  neuropathic  tendency  in 
the  family,  viz.  cases  of  epilepsy,  imbecility,  insanity.  In  Milroy's  case 
he  traced  twenty-two  individuals  among  ninety-seven  persons  in  six 
generations. 

Treatment. — Continuous  bandaging  is  the  only  measure  that  is  capable 
to  give  relief. 

ERYTHROMELALGIA 

It  is  characterized  by  reddening  of  the  skin  and  paroxysmal  pain  in 
the  feet  and  sometimes  in  the  hands.  It  was  first  described  and  named 
by  Weir-Mitchell  in  1878. 

Symptoms. — The  feet  are  more  frequently  affected  than  the  hands. 
Pain  is  the  first  symptom  to  appear.  The  great  toe  is  particularly  painful 
at  the  beginning.  The  pain  occurs  in  paroxysms  and  later  the  intervals 
become  shorter  and  shorter.  The  pain  is  excruciating  and  is  relieved  by 
recumbent  position  and  cold.  Station,  gait,  pressure  or  heat  and  exercise 
increase  it.     When  the  hands  are  affected,   the  patient  gets  relief  by 


586  ERYTHROMELALGIA 

crossing  the  arms  over  the  breast  or  by  raising  them  above  the  head. 
Such  an  attitude  removes  the  blood  from  the  periphery. 

Soon  redness  of  the  skin  develops.  At  first  it  is  rosy-red,  but  later 
purplish-red.  It  is  especially  marked  on  the  last  phalanges.  The  veins 
are  distended  and  a  swelling  is  noticeable.  The  local  temperature  is 
increased  and  a  hyperhidrosis  takes  place.  The  sensations  may  be  di- 
minished or  increased. 

While  in  the  majority  of  cases  the  distal  ends  of  the  limbs  are  involved, 
nevertheless  the  pain  and  redness  may  sometimes  extend  to  the  entire 
limb.  The  symptoms  may  be  observed  also  on  other  parts  of  the  body, 
viz.  face.,  ears,  testicles,  chest,  back,  also  in  rare  cases  in  the  mucous 
membranes  of  the  mouth  and  throat  (Seeligmiiller.) 

Other  Symptoms. — During  the  paroxysms  headache,  vertigo,  tinnitus 
aurium  and  even  syncope  may  occur.  Hardening  of  subcutaneous 
tissue,  thickening  of  the  nails,  also  oedema  occasionally  occur.  Atrophy  of 
the  muscles  of  the  extremities  may  develop  in  advanced  cases.  A  dim- 
inution or  increase  of  electrical  contractility  has  been  observed,  but  no 
reactions  of  degeneration. 

Erythromelalgia  is  sometimes  observed  in  the  course  of  organic  or 
functional  nervous  diseases,  also  in  diseases  of  peripheral  nerves. 

Course,  Duration,  Prognosis.— Amelioration  and  aggravation  in  the 
course  of  the  disease  are  observed.  It  lasts  an  indefinite  time.  The 
prognosis  is  unfavorable,  as  the  disease  is  rebellious  to  treatment. 

Diagnosis. — The  cyanosis,  local  elevation  of  temperature,  swelling,  are 
sufficiently  characteristic  symptoms  for  diagnosis. 

Erythromelalgia  should  be  differentiated  from  angioneurotic  oedema, 
acroparaesthesia  and  initial  stages  of  Raynaud's  disease  (see  these  chapters). 
Mention  should  be  made  of  so-called  incomplete  or  allied  forms  of 
erythromelalgia.  I  observed  a  patient  whose  affection  began  with  red- 
ness of  the  hands  and  the  pain  appeared  only  later;  cold  would  increase 
the  pain;  exercise  had  no  effect  upon  1he  pain.  It  is  therefore  a  case 
which  apparently  belongs  to  the  group  of  ervthromelalgia  and  yet  not 
typical. l 

Etiology. —  Cold,  physical  fatigue,  are  predisposing  factors.  Infec- 
tions, rheumatism,  syphilis  and  accompanying  nervous  diseases,  such  as 
hemiplegia,  cerebral  tumors,  multiple  neuritis,  neuralgia,  hysteria, 
neurasthenia,  etc.,  are  the  main  causes. 

Pathogenesis. — The  majority  of  observers  consider  the  disease  as  a 
vaso -motor  neurosis.  Whether  it  is  due  to  a  paresis  of  the  vasoconstrict- 
ors or  to  an  irritation  of  the  vaso-dilators,  it  is  difficult  to  say.     According 

1  American  Medicine,  August,  1907. 


RAYNAUD'S    DISEASE  587 

to  some  the  spinal  gray  matter  is  the  source  of  the  disease.  Others  believe 
in  a  disease  of  the  peripheral  arteries.  Weir-Mitchell  thinks  that  there  is 
a  neuritis  of  the  sensory  nerves. 

Treatment. — Electricity,  application  of  cold  and  sedatives  for  relief 
of  pain  are  the  only  measures  employed.  Perhaps  Bier's  method  of 
artificial  hyperasmia  may  render  some  service.  If  syphilis  is  suspected, 
and  even  without  evidences  of  syphilis,  mercury  and  iodides,  also  salvarsan 
may  be  tried.  Removal  of  small  portions  of  nerves  or  stretching  of  nerves, 
also  amputations  of  toes  or  fingers,  are  to  be  attempted  as  a  last  resort. 

RAYNAUD'S  DISEASE 

It  is  characterized  by  an  ischemia  or  a  hyperemia  of  the  extremities 
which  may  terminate  in  a  symmetrical  gangrene.  It  was  described  by 
Raynaud  in  1862. 

Symptoms. — The  disease  presents  three  phases.  The  first  is  char- 
acterized by  local  ischemia,  the  second  by  a  passive  hyperemia,  the 
third  by  gangrene. 

First  Phase. — Suddenly  the  fingers  or  toes  or  in  exceptional  cases  the 
entire  hand  or  foot  become  pale,  wax-like  and  cold.  This  is  accompanied 
or  preceded  by  a  numbness,  tingling  or  severe  pain.  The  condition  may 
disappear  rapidly  or  be  followed  by  a  cyanosis  (second  phase).  In  the 
latter  case  the  skin  becomes  blue.  Then  the  pain  increases.  The  part 
is  swollen  but  not  cedematous.  The  anemia  produced  by  pressure  with 
a  finger  is  slowly  obliterated.  The  temperature  is  lowered.  The  pallor 
alternating  with  cyanosis  (local  asphyxia)  or  cyanosis  without  the  pre- 
ceding pallor,  when  repeated  may  become  very  frequent  and  then  the 
patient  enters  into  the  third  phase  of  the  disease,  characterized  either  by 
active  hyperemia  or  gangrene.  The  blue  color  of  the  skin  turns  red. 
The  part  gets  hot  and  throbs.  This  period  lasts  a  variable  time.  It 
may  be  entirely  missing.  The  asphyxia  may  persist  and  the  circulation 
not  be  reestablished.  In  such  a  case  the  final  period  of  gangrene  is  to  be 
feared.  Gangrene  may  follow  even  the  first  stage  of  syncope :  in  such  a  case 
the  parts  never  recover  the  circulation.  Dark  spots  and  vesicles  appear; 
the  serum  of  the  latter  dries  up  and  a  crust  forms.  When  the  latter  falls 
off,  an  ulcerated  surface  is  noticed  which  is  slow  in  healing. 

The  gangrene  may  extend  and  involve  an  entire  phalanx.  The  skin 
is  parchment-like,  the  phalanx  mummifies,  a  line  of  demarcation  is  formed 
and  gradually  the  gangrenous  part  becomes  detached.  Cicatrization  of 
the  stump  may  be  rapid  or  slow.  During  the  entire  process  fever  is  absent, 
but  the  pain  is  excruciating.  The  suffering  disturbs  the  patient's  sleep, 
digestion  and  the  general  nutrition. 


588  Raynaud's  disease 

The  phase  of  "pallor"  and  that  of  "cyanosis"  correspond  to  "Ray- 
naud's "local  syncope"  and  "local  asphyxia,"  respectively.  They  are, 
properly  speaking,  two  successive  stages  of  the  disease  and  they  eventu- 
ally terminate  in  the  final  phase  of  symmetrical  gangrene.  However,  the 
first  two  periods  may  exist  a  long  time  conjointly  or  separately  without 
entering  the  final  stage.  Also  the  local  syncope  may  exist  a  long  time 
before  the  second  period  makes  its  appearance. 

Local  syncope  occurs  in  attacks  lasting  but  a  few  minutes  or  several 
hours.  The  patient  complains  of  "dead  fingers."  The  attacks  recur 
sometimes  with  great  regularity.  They  may  occur  either  daily  for  some 
time  or  every  two  or  three  months.  They  are  usually  precipitated  by 
exposure  to  cold. 

The  place  of  local  asphyxia  (cyanosis)  is  mostly  preceded  by  attacks 
of  local  syncope  and  it  may  last  days,  weeks  or  months.  In  mild  cases 
it  may  disappear,  but  in  severer  cases  it  is  followed  by  the  phase  of  sym- 
metrical gangrene. 

Other  Symptoms. — Objective  sensations  are  usually  decreased. 
Anaesthesia  is  common  in  the  period  of  syncope.  Parassthesia  is  present 
in  the  period  of  asphyxia.  Hyperassthesia  is  usually  present  after  a 
paroxysm.  Atrophy  of  the  neighboring  muscles  with  impairment  of 
motion  is  not  infrequent.  Pupillary  contraction,  tinnitus  aurium,  albu- 
minuria, may  occur.     Mental  depression  is  a  frequent  accompaniment. 

Not  only  the  fingers  and  toes,  but  also  the  heels,  coccyx,  the  ears, 
nose  and  the  prominent  bones  of  the  cheeks  may  be  affected. 

The  disease  is  usually  symmetrical  and  bilateral,  although  unilater- 
ality  has  been  observed. 

Complications. — Panas  reports  a  case  in  which  there  was  a  relation 
between  the  cyanotic  attacks  and  the  condition  of  the  arteries  in  the 
fundus  oculi:  when  cyanosis  occurred,  the  arteries  were  contracted;  when 
the  former  subsided,  the  latter  became  normal. 

In  some  cases  Raynaud's  disease  was  preceded  or  followed  by  attacks 
of  transient  hemiplegia,  monoplegia,  aphasia  or  amblyopia.  Epileptic 
convulsions  have  been  observed  in  association  with  attacks  of  local 
cyanosis.     Hemoglobinuria  is  a  rare  complication. 

Purpura  and  urticaria  have  been  exceptionally  observed. 

Course,  Duration,  Prognosis. — The  course  may  consist  only  of  the 
first  or  of  the  first  and  second  phases.  More  frequently  the  entire  cycle 
is  observed.  The  duration  of  the  disease  is  from  one  to  three  months. 
After  an  interval  of  months  or  years  another  attack  may  occur  and  affect 
another  part  of  the  body.  Life  is  usually  not  endangered,  except  when 
suppuration  with  septic  infection  is  present. 


RAYNAUD  S    DISEASE 


589 


Diagnosis. — The  disease  is  easily  recognized  from  its  typical  symptoms. 
Local  gangrenous  patches  may  also  be  observed  in  hysteria,  but  the 
necrosis  is  superficial  and  no  changes  of  the  blood  vessels  are  found. 

In  lepra  phalanges  fall  off  without  pain.  Gangrene  caused  by  in- 
toxication with  ergot  presents  special  features  characteristic  of  ergotism. 

I  saw  a  case  of  local  gangrene  caused  by  subcutaneous  injections  of 
adrenalin  above  the  lesion. 

There  are  also  allied  cases  in  which  the  picture  of  Raynaud's  disease 
is  not  complete,  cases  that  present  intermediary  forms,  which  may  resemble 


Fig.  168. — Symmetrical  Gangrene.     (Dehio). 

one  or  another  of  the  localized  neuroses.  In  the  case  referred  to  in 
the  chapter  on  Erythromelalgia  the  symptoms  were  those  of  Raynaud's 
disease  and  of  erythromelalgia. 

Etiology. — The  disease  occurs  not  infrequently  in  the  course  of  organic 
nervous  diseases  and  psychoses,  also  in  functional  nervous  disorders. 
Infectious  febrile  diseases,  syphilis,  tuberculosis,  lepra,  diabetes,  may  be 
accompanied  or  followed  by  Raynaud's  disease.  It  has  been  observed 
in  the  course  of  scleroderma.  Anaemia  and  a  congenitally  small  aorta 
play  an  important  predisposing  part. 

Emotion,  trauma,  menses,  cold,  are  exciting  causes.  Zenner  observed 
a  case  due  to  caffein  poisoning.  Young  persons,  and  especially  women, 
are  more  frequently  affected  than  old  individuals  and  men. 

Pathogenesis. — Pathological  investigations  show  a  localized  involve- 
ment of  the  blood  vessels  (arteritis)  and  of  the  nerves  (neuritis),  but  these 
changes  are  secondary.  The  majority  of  observers  consider  Raynaud's 
disease  a  vasomotor  neurosis  (see  also  erythromelalgia).  Raynaud 
believed  that  the  vaso-motor  centers  are  in  a  state  of  irritability;  the 


590  RAYNAUD  S    DISEASE 

irritant  comes  from  the  periphery  (cold),  the  paroxsysmal  contraction  of 
the  blood  vessels  comes  from  the  center. 

Treatment. — Rest  and  local  applications  of  heat  are  important. 
Good  hygienic  and  dietetic  measures  are  necessary. 

Pain  can  be  combated  by  usual  means,  such  as  coal-tar  products, 
etc.  Amylnitrite  in  inhalations  during  the  attacks  may  give  relief  from 
pain,  but  it  is  not  always  effective.  Nitroglycerine  and  ergot  have  also 
been  recommended.  Bier's  method,  consisting  of  an  application  of 
bandage  above  the  affected  parts  for  an  hour  twice  daily,  will  induce  an 
artificial  hyperaemia  and  give  relief.  It  may  be  of  benefit  in  the  first  two 
phases  of  the  affection.  Cushing's  method  may  be  very  useful.  He 
bandages  lightly  the  limb,  then  applies  a  tourniquet  for  a  minute  or  two. 
When  the  latter  is  removed,  a  large  amount  of  blood  rushes  to  the  parts 
which  were  made  anaemic.     It  may  be  tried  several  times  a  day. 

Barlow  recommends  the  following  procedures.  "Immerse  the  limb 
into  a  basin  containing  salt  and  tepid  water;  one  pole  of  a  constant 
current  battery  is  placed  in  contact  with  the  upper  part  of  the  limb 
above  the  level  of  the  water,  the  other  pole  in  the  basin.  The  current  is 
then  made  and  broken  at  frequent  intervals  so  as  to  obtain  moderate 
contractions  of  the  limb.  The  patient  is  also  told  to  move  the  digits 
while  electricity  is  being  applied."  He  claims  that  considerable  relief  is 
obtained  in  cases  of  recurrent  local  asphyxia.  High-frequency  current 
has  also  been  recommended.  In  case  of  gangrene  antiseptic  applica- 
tions with  rest  of  the  limbs  are  the  only  measures  besides  operative 
considerations. 


CHAPTER  XXVIII 

NERVOUS  SYMPTOMS  PRODUCED  BY  INTOXICATIONS 

A.  METALLIC  POISONS 
I.  Lead  Intoxication 

Lead  may  affect  the  central  and  the  peripheral  nervous  system,  also 
produce  psychoses. 

Pathology. — A  striking  and  at  the  same  time  a  common  feature  is 
arteriosclerosis.  It  is  not,  however,  conclusively  proven  that  lead  is  the 
direct  cause  of  the  thickening  of  the  vessel  walls.  In  the  brain  the 
meninges  are  altered:  pachymeningitis,  leptomeningitis  with  oedema  and 
atrophy  of  the  cerebral  tissue  are  frequently  observed.  Changes  of  the 
cortical  cells,  proliferation  of  the  nuclei  in  the  vessel  walls  and  of  the 
glia-cells  about  the  vessels  have  been  found.  In  the  cord  chronic  anterior 
poliomyelitis  may  occur.  In  a  case  under  my  observation  I  found 
microscopically  degeneration  of  the  posterior  columns  {American  Medicine, 
1905).  The  peripheral  nerves  present  parenchymatous  degeneration. 
For  details  see  the  chapter  on  multiple  neuritis. 

Symptoms,  (a)  Cerebral  Encephalopathy. — In  acute  poisoning  head- 
ache, vertigo,  epileptiform  convulsions,  delirium  and  hallucinations, 
also  coma,  are  observed.  The  chronic  form  may  be  accompanied  by 
persistent  headache,  also  convulsions,  attacks  of  hemiplegia,  attacks  of 
aphasia,  finally  by  psychoses  (see  below). 

Functional  nervous  diseases  may  be  induced  by  chronic  lead  poisoning. 
Choreiform  movements,  hysterical  hemianaesthesia  have  been  observed. 
Generalized  or  focal  epilepsy  are  not  infrequent. 

The  psychoses  due  to  lead  may  present  themselves  in  the  form  of  a 
delirium  or  more  frequently  by  stupor.  Delirium  usually  lasts  weeks. 
Symptoms  of  paresis  are  not  rare,  but  they  do  not  present  the  typical 
picture  of  this  psychosis.  The  condition  is  therefore  called  "pseudo- 
paresis"  (see  Paresis).  Delusions  of  persecution  with  terrifying 
hallucinations  occur.  The  latter  are  particularly  present  in  cases  which 
are  at  the  same  time  alcoholic. 

Eye  Symptoms.- — Palsies  of  the  ocular  muscles,  also  nystagmus, 
may  occur.     Hemianopsia,  usually  homonymous,  amaurosis  are  usually 

59i 


592  NERVOUS    SYMPTOMS    PRODUCED   BY    INTOXICATIONS 

transitory.  In  some  cases  profound  changes  in  the  optic  nerves  take 
place. 

(b)  Cord  Symptoms.— Muscular  atrophy  due  to  poliomyelitis  is  not 
rare.  Aran-Duchenne's  type  of  atrophy  with  the  claw-like  hand  is  oc- 
casionally seen.  Erb's  type  has  also  been  observed  (see  Progressive 
Muscular  Atrophy). 

(c)  Peripheral  Nerve  Symptoms. — See  chapter  on  Multiple  Neuritis. 
Course,  Duration,  Prognosis. — Cerebral  symptoms  present  a  serious 

outlook.  When  optic  neuritis  is  established,  the  prognosis  is  doubtful. 
Blindness  may  be  the  outcome.  When  cardiac  or  renal  complications 
occur,  the  prognosis  is  grave.  In  most  favorable  cases  the  prognosis 
should  be  guarded,  as  recurrences  are  not  infrequent.  Finally  the 
patient's  future  will  depend  upon  whether  he  is  removed  from  the  influence 
of  lead  or  not. 

Etiology. — The  occupations  of  painters,  compositors,  occupations  re- 
quiring a  free  handling  of  lead  and  lead  colors,  especially  lead  carbonate 
in  a  dry  state,  the  use  of  certain  cosmetics,  the  use  of  water  kept  in  lead 
vessels,  are  the  usual  sources  of  lead  intoxication. 

Treatment. — Elimination  of  lead  is  the  first  indication.  Purgatives 
and  diuretics  are  necessary.  In  view  of  the  obstinate  constipation 
characteristic  of  lead  poisoning,  moderate  doses  of  atropine  should  be 
given  with  the  purgatives  in  order  to  control  the  intestinal  spasm.  To 
enhance  diuresis  large  quantities  of  water  are  to  be  given.  Baths  of 
potassium  sulphuret  will  help  elimination  of  lead:  to  six  inches  depth 
of  water  six  ounces  of  potassium  sulphuret  are  added.  Potassium  or 
sodium  iodide  internally  is  excellent.  Exclusive  milk  diet  at  first  and 
later  milk  and  vegetable  diet,  hydrotherapy,  avoidance  of  stimulants, 
including  tea  and  coffee,  are  the  main  elements  of  the  treatment.  In- 
somnia, headache,  gastric  disturbances,  convulsions,  are  treated  accord- 
ingly. Venesection  and  lumbar  puncture  may  be  useful  in  cases  of 
cerebral  congestion. 

Paralysis  is  to  be  treated  with  massage  and  electricity. 

II.  Arsenical  Intoxication 

Pathology. — No  special  lesion  has  been  found  in  the  brain.  In  the 
spinal  cord  there  may  be  involvement  of  gray  and  white  matter  (myelitis). 
Changes  in  the  peripheral  nerves  are  the  most  important.  See  Multiple 
Neuritis. 

Symptoms. — In  chronic  cases  there  may  be  present  cerebral  symp- 
toms, viz.  impairment  of  memory,  of  intellectual  faculties.  In  acute 
cases  delirium  and  confusion  are  the  usual  manifestations. 


NERVOUS    SYMPTOMS    PRODUCED   BY    INTOXICATIONS  593 

Eye  symptoms  may  be  amblyopia  or  amaurosis,  ocular  palsies.  Puf- 
finess  of  the  eyelids,  congestion  of  the  conjunctivae,  are  quite  common. 

When  the  cord  is  involved,  symptoms  of  myelitis  are  evident. 

For  the  symptoms  of  arsenical  neuritis  see  chapter  on  Multiple 
Neuritis. 

Course,  Duration,  and  Prognosis  are  usually  favorable  and  similarly 
to  all  toxic  conditions  may  be  serious,  if  the  intoxication  is  severe  and 
protracted. 

Etiology. — Wall  paper,  colored  dress  goods,  arsenical  paint,  rugs, 
carpets,  crayons,  beer  (glucose  in  England),  medicinal  arsenic  and  finally 
intentional  absorption  are  all  sources  for  intoxication. 

Treatment. — See  preceding  chapter. 

III.  Mercurial  Intoxication 

Pathology.- — Cerebral  oedema,  degenerative  changes  in  the  tracts 
of  the  cord  and  in  its  gray  matter,  atrophy  of  the  nerve  fibers,  sclerotic 
changes  of  the  vessel  walls,  are  the  usual  findings. 

Symptoms — Cerebral  excitement  or  else  depression,  vertigo,  tremor, 
choreiform  movements,  convulsions,  muscular  weakness,  disturbance  of 
speech  (caused  by  tremor  of  lips),  diminution  or  loss  of  cutaneous 
sensibility,  amblyopia  and  sometimes  optic  neuritis  are  the  chief  symp- 
toms. In  advanced  cases  impairment  of  memory,  confusion  and  mild 
dementia  may  develop.  Tremor  is  the  most  conspicuous  symptom.  At 
first  it  is  very  slight.  Soon  it  becomes  marked  upon  the  least  emotion. 
The  range  of  the  tremor  may  be  small  or  gross.  While  at  first  it  is 
present  only  on  voluntary  acts,  later  it  appears  even  when  the  patient  is 
at  rest.  The  hands  and  lips  are  affected  first,  but  later  all  extremities 
and  face  become  involved.  The  twitchings  may  sometimes  be  very 
violent. 

In  the  chapter  on  Multiple  Neuritis  details  are  given  concerning  the 
manifestations  of  the  peripheral  nerve  involvement. 

Course,  Duration,  Prognosis. — See  the  previous  chapter. 

Etiology. — Manufacture  of  mirrors,  of  rubber,  the  use  in  paints,  in 
artificial  flowers,  in  aniline  colors,  in  fireworks,  in  mining,  in  smelting,  in 
manufacture  of  thermometers,  finally  the  use  for  medicinal  purposes  are 
all  the  usual  sources  of  mercurial  poisoning. 

Treatment. — See  preceding  chapter. 

IV.  Carbon  Monoxide  Intoxication 

Acute  poisoning  may  be  followed  immediately  or  after  a  considerable 
time  by  persistent  disturbances  in  the  nervous  system. 
38 


594  NERVOUS    SYMPTOMS    PRODUCED   BY    INTOXICATIONS 

Pathology. — Diffuse  encephalomyelitis  is  the  characteristic  feature 
of  the  changes  in  the  central  nervous  system.  Hyperemia,  hemorrhages 
in  the  cortex,  in  the  basal  nuclei,  in  the  internal  capsule,  foci  of  soften- 
ing particularly  in  the  lenticular  nuclei  (see  Diseases  of  Basal  ganglia), 
poliomyelitic  foci  in  the  cord,  peripheral  neuritis,  are  all  conditions  met 
with. 

Symptoms. — Immediately  after  the  poisoning  the  following  nervous 
disturbances  are  observed:  headache,  vertigo,  nausea,  vomiting,  muscular 
weakness,  relaxation  of  sphincters  and  loss  of  consciousness.  Muscular 
twitching  and  even  convulsions  may  occur. 

If  consciousness  is  regained,  there  is  a  marked  mental  hebetude. 

The  permanent  nervous  symptoms  are :  neuritis,  neuralgias,  anaesthe- 
sias, choreiform  movements,  intention  tremor  and  scanning  speech.  For 
details  on  neuritis  consult  the  chapter  on  Multiple  Neuritis.  Ocular 
palsies,  nystagmus,  partial  or  complete  blindness  may  occur. 

Paralysis,  hemiplegia  or  monoplegia  and  mental  disturbances  are  not 
rare.  They  may  develop  weeks  after  the  poisoning.  Amnesia  is  quite 
common.  In  my  case  {New  York  Medical  Jour.,  1906)  there  was  persist- 
ent antero-retrograde  amnesia.  Confusion  or  stupor  with  or  without 
hallucinations  may  last  from  weeks  to  two  or  three  months.  The  para- 
lytic symptoms  may  be  permanent.  Cases  with  total  dementia  have  been 
recorded.  All  these  sequelae  may  appear  immediately  or  weeks  after  the 
poisoning.  It  is  important  to  know  that  sometimes  very  serious  sequelae 
develop  in  cases  of  mild  poisoning. 

Course,  Duration,  Prognosis. — Recovery  may  follow  in  a  very  short 
time.  It  depends  frequently  upon  the  promptness  with  which  treatment 
is  instituted.  The  prognosis  should  be  guarded  in  view  of  the  sequelae 
that  may  follow  some  time  after  the  poisoning.  Cerebral  manifestations 
are  usually  of  grave  omen. 

Etiology. — Illuminating  gas,  gas  works,  furnaces,  heating  apparatuses 
with  poor  draughts,  tar  distilleries,  chemical  factories,  laboratories,  are 
the  sources  of  carbon  monoxide  poisoning. 

Treatment. — Removal  from  the  poisoned  atmosphere,  inhalation  of 
oxygen,  venesection  followed  by  injection  of  normal  salt  solution,  ad- 
ministration of  stimulants,  are  the  immediate  measures.  Prophylaxis  is 
the  most  important  feature. 

V.  Manganese  Intoxication. 

L.  Casamajor  has  very  recently  (/.  Am.  Med.  Assn.,  1913)  called 
attention  to  a  symptom-group  produced  by  poisoning  with  manganese. 
The  cases  that  came  under  his  observation  occurred  among  workers  in  the 


NERVOUS  SYMPTOMS  PRODUCED  BY  INTOXICATIONS         595 

separating-mill  connected  with  a  mine  from  which  zinc,  iron  and  manganese 
are  the  principal  products.  The  handling  of  the  ground  ore  is  done  in  a 
dry  state;  the  air  which  the  workmen  breathe  is  laden  with  a  very  fine 
gray  dust. 

As  zinc  and  iron  have  never  produced  a  poisoning  similar  to  the  one 
under  discussion,  the  author  concludes  that  manganese  is  very  probably 
the  etiologic  factor. 

Symptoms. — A  man  working  in  this  dust  for  a  period  of  six  months  or 
more  begins  to  notice  a  difficulty  of  walking  down  an  incline.  If  he  does, 
he  must  run  fast  until  he  meets  an  object  (propulsion).  Walking  up  a  hill 
or  on  a  level  ground  is  normal.  Walking  backward  is  impossible  and  if  an 
attempt  is  made,  the  patient  falls  (retropulsion).  In  addition  to  the 
disturbance  of  gait,  the  patient  may  have  pain  and  stiffness  in  the  legs. 
The  picture  resembles  that  of  paralysis  agitans. 

Other  Symptoms.- — Defective  hearing,  slurring  speech,  mask-like 
f  acies,  tremor  of  the  tongue  and  hands,  unsteady  station.  These  symptoms 
were  not  found  simultaneously  in  all  the  patients.  Sensations,  reflexes, 
sphincters,  are  all  normal. 

Course,  Prognosis. — The  course  is  chronic.  When  removed  from  the 
dust  the  patient  may  improve,  but  this  is  not  in  every  case.  The  outlook 
is  poor. 

Pathology. — In  one  case  that  came  to  autopsy  the  following  condition 
was  found.  Perivascular  spaces  of  the  lenticular  nuclei  and  of  the  outer 
part  of  optic  thalami  are  larger  than  normal.  No  other  gross  changes 
have  been  observed. 


B.     ORGANIC  POISONS 

I.  Alcoholic  Intoxication.    Alcoholism 

Alcohol  has  a  special  predilection  for  the  nervous  system.  Its  effect 
upon  the  nervous  system  differs  in  acute  and  chronic  intoxications. 

Acute  Alcoholism. — Mental  symptoms  are  predominating.  Delirium, 
confusion  and  stupor  with  or  without  hallucinations  and  illusions  are  the 
characteristic  features.  They  are  fugacious,  transitory.  Recovery 
usually  follows.  The  promptness  of  the  recovery  depends  upon  the  effect 
of  alcohol  on  different  individuals.  When  the  alcoholic  abuse  is  frequently 
repeated,  a  state  of  chronic  alcoholism  develops. 

Chronic  Alcoholism. — When  the  absorption  of  alcohol  is  slow  and 
prolonged,  the  changes  it  produces  in  the  individual's  physical  and 
mental  spheres  of  life  are  enormous. 


596        NERVOUS  SYMPTOMS  PRODUCED  BY  INTOXICATIONS 

Physical  Symptoms. — Gastro-intestinal  disturbances  are  marked  by 
anorexia,  vomiting,  constipation  or  else  diarrhoea  with  bloody  discharge. 

Tremor  is  common.  It  is  passive  and  intentional.  It  affects  the 
extremities  as  well  as  the  tongue  and  lips. 

Epileptic  convulsions  are  not  rare.  They  may  be  unilateral  or 
generalized. 

Attacks  of  apoplexy  followed  by  paralysis  occur. 

Neuritis  is  very  common.  For  details  see  chapter  on  Multiple 
Neuritis. 

Vaso-motpr  disturbances,  increased  mechanical  irritability  of  muscles 
and  nerves,  various  subjective  disturbances,  as  pains,  coldness,  numbness, 
palpitation,  fears,  general  weakness,  are  all  accompanying  symptoms. 

Ocular  disturbances  are  not  infrequent.  They  are:  contraction  of  the 
visual  field,  scotomata,  optic  atrophy  and  optic  neuritis. 

Mental  Symptoms. — In  my  series  of  277  cases  of  chronic  alcoholism 
(/.  of  Am.  Med.  Assn.,  1907)  the  mental  disturbances  followed  repeated 
subacute  attacks  or  repeated  attacks  of  delirium  tremens,  but  also  in- 
sidiously and  progressively  without  preliminary  acute  symptoms.  The 
mental  state  consists  chiefly  of  a  gradually  developing  intellectual  feeble- 
ness, viz.  dementia.  Before  the  latter  becomes  conspicuous,  the  patient 
begins  to  show  undue  irritability  which  at  first  is  noticeable  only  to  the 
immediate  surroundings.  At  the  same  time  appears  a  weakness  of  the 
will  power  and  of  energy.  The  patient  soon  becomes  depressed,  his 
memory  clouded,  the  power  of  application  for  work,  physical  or  cerebral, 
decidedly  impaired.  The  sadness,  the  realization  of  his  physical  and 
mental  impotence,  lead  him  directly  to  delusive  ideas  which  become 
intensified  by  hallucinatory  images,  and  criminal  tendencies  are  not 
infrequently  observed.  Gradually  the  moral  sense,  the  sense  of  propriety, 
becomes  deteriorated,  conventional  laws  are  totally  ignored.  The 
patient  becomes  apathetic,  brutal.  His  cerebral  functions  are  totally 
disorganized,  the  judgment  infantile  and  dementia  is  permanently 
established. 

An  acute  episode  in  the  course  of  chronic  alcoholism  is  seen  in 

Delirium  Tremens. — As  the  name  implies,  it  is  characterized  by  a 
delirious  state  and  tremor.  The  condition  usually  develops  gradually. 
At  first  there  is  a  state  of  restlessness,  insomnia  with  hallucinations; 
tremor  appears  in  the  hands  and  tongue.  These  few  symptoms  continue 
to  increase  in  intensity.  The  patient  becomes  very  restless,  very  talkative, 
attempts  to  get  up,  if  he  is  in  bed,  runs  from  place  to  place;  talks  aloud  and 
appears  to  converse  with  imaginary  persons.  His  actions  are  prompted 
by  various  hallucinatory  images.     Through  the  visual  apparatus  he  sees 


NERVOUS  SYMPTOMS  PRODUCED  BY  INTOXICATIONS         597 

terrifying  animals,  assassins,  robbers,  executions,  fires,  etc.  Through  the 
auditory  apparatus  he  hears  threats,  oaths,  bad  names,  etc.  Through 
olfactory  apparatus  he  perceives  the  most  repugnant  odors.  Through 
the  gustatory  apparatus  he  tastes  nauseating  substances.  Through  the 
sense  of  touch  he  believes  himself  undergoing  torture.  Being  under  the 
influence  of  these  hallucinations  the  patient  is  excited,  defends  himself, 
threatens,  strikes  or  else  is  terrified  and  lays  immobile.  Orientation  in 
time  and  space  is  frequently  affected.  A  patient,  for  example,  may  re- 
member the  objects  of  his  room,  but  does  not  recognize  that  he  is  in 
his  own  room.  A  feeling  of  great  anxiety  is  ever  present  because  of  the 
hallucinations,  but  when  the  patient  reaches  the  climax  of  his  delirium, 
the  anxiety  is  considerably  diminished  and  may  even  disappear.  He  then 
becomes  indifferent  to  the  hallucinations. 

These  mental  phenomena  are  accompanied  by  a  tremor  affecting  the 
hands  or  the  upper  and  lower  extremities  or  the  latter  and  the  face.  In 
some  cases  the  tremor  continues  even  in  sleep.  The  speech  shows  an 
incoordination:  stumbling  over  syllables  and  words,  difficulty  in  pronun- 
ciation. The  loss  of  appetite  is  striking  during  the  delirious  state.  The 
pulse  runs  from  80  to  115  and  is  dicrotic.  The  temperature  is  usually 
normal,  but  it  may  rise  as  high  as  1030  and  even  1050.  Constipation  is  the 
rule.  Albumen  is  present  in  the  majority  of  cases.  The  polynuclear 
leucocytes  of  the  blood  are  increased  and  the  mononuclear  are  decreased 
(Elsholz). 

The  duration  of  an  attack  is  usually  from  two  to  eight  days,  according 
to  its  intensity.  It  usually  ends  in  a  deep  sleep  lasting  from  twelve  to 
thirty  hours.  When  the  patient  awakens,  the  delirium  and  hallucinations 
are  gone,  orientation  is  good,  but  some  mental  hebetude  remains,  the  mem- 
ory is  somewhat  cloudy  and  a  slight  tremor  persists. 

Alcoholic  serous  meningitis  (wet  brain)  is  quite  frequent  in  chronic 
alcoholism.  It  usually  follows  an  attack  of  delirium  tremens.  In  such 
a  case  the  patient  from  the  onset  enters  into  semi-comatous  state.  Delir- 
ium is  present  but  not  of  a  violent  nature.  The  patient  though  affected 
by  hallucinations  lies  motionless  and  mutters  in  a  low  voice.  He  can 
hardly  be  roused  for  food.  The  pulse  is  rapid,  temperature  normal. 
The  pupils  are  small.  Hyperesthesia  is  marked.  Recovery  is  possible 
especially  in  those  cases  in  which  the  neck  is  not  rigid  (Dana).  In  cases 
with  rigid  neck  Dana  believes  the  patients  die.  In  such  cases  the  semi- 
comatose state  gets  deeper  and  deeper,  general  rigidity  develops,  the 
abdomen  gets  retracted.  If  recovery  occurs,  the  condition  normally  lasts 
about  four  weeks. 

The  prognosis   depends  upon  whether   complicating  conditions  are 


598  NERVOUS    SYMPTOMS    PRODUCED   BY   INTOXICATIONS 

present  or  not.  It  is  grave  in  cases  of  trauma,  infectious  diseases,  neph- 
ritis, cardiac  weakness,  pneumonia. 

Recurrences  are  not  infrequent.  An  attack  may  come  on  from  some 
insignificant  cause,  as  slight  trauma  or  an  ingestion  of  a  small  amount  of 
alcohol  after  a  period  of  abstinence,  or  else  from  an  intercurrent  febrile 
disease. 

Pathology  of  Alcoholism. — Little  or  no  special  changes  are  found  in 
acute  alcoholism. 

In  chronic  alcoholism  the  most  constant  alterations  are  observed  in 
the  blood  vessels.  Atheromatous  changes  of  the  minute  blood  vessels 
are  conspicuous.  They  are  enlarged,  tortuous.  In  their  vicinity  neuroglia 
cells  are  abundant.  The  perivascular  spaces  are  filled  with  lymphoid 
cells.  Pachymeningitis  and  leptomeningitis,  oedema  of  the  brain,  pachy- 
meningitis hemorrhagica  are  all  quite  frequent.  Degeneration  of  the 
cortical  cells,  especially  in  the  motor  areas;  also  of  the  descending  tracts 
in  the  internal  capsule  and  in  the  spinal  cord;  finally  in  the  cells  of 
the  anterior  cornua  in  the  cord  have  been  repeatedly  found.  In  delirium 
tremens  there  is  a  great  tendency  to  minute  hemorrhages  especially  in 
frontal  and  central  convolutions  of  the  brain,  frequently  also  in  the  gray 
matter  of  the  aqueduct  of  Sylvius  and  around  the  third  ventricle.  As  to 
the  peripheral  nerves,  they  particularly  are  affected  by  alcohol  (see 
chapter  on  Multiple  Neuritis).  Korsakoff's  psychosis  is  described  in 
connection  with  Alcoholic  Multiple  Neurit's. 

Treatment. — Withdrawal  of  the  poison  is  the  first  indication.  Some 
believe  in  gradual  reduction  of  the  beverage,  others  in  sudden  removal. 
This  can  be  well  accomplished  by  isolation.  Sleep  must  be  induced  by 
all  means.  Bromides  (twenty*  grains),  paraldehyde  (one  dram),  chloral 
hydrate  (30-60  grains),  sulphonal  (gr.  x),  trional  (gr.  x),  veronal  (gr.  x) 
are  good  drugs  for  insomnia.  Morphia  (gr.  1/8)  associated  with  chloral  (gr. 
xx)  is  especially  advantageous.  A  tepid  bath  of  hah  an  hour's  duration 
is  also  useful  in  insomnia.  As  withdrawal  of  alcohol  may  cause  collapse 
or  exhaustion,  a  hypodermic  injection  of  ergot  may  be  very  efficient.  In 
case  the  patient  is  exceedingly  restless,  or  violent,  a  hypodermic  injec- 
tion of  apomorphine  (gr.  1/10)  is  advisable. 

After  sleep  is  secured,  the  regular  treatment  will  consist  of  a  complete 
or  partial  rest,  moderate  outdoor  exercises,  regular  meals,  hydrotherapy 
and  internal  administration  of  strychnia.  Milk  should  be  the  main 
article  of  food.  Meat  is  to  be  avoided.  Purgatives  (preferably  saline) 
are  useful  when  given  two  or  three  times  a  week.  Coffee  and  tea  are  to 
be  avoided. 

Bromides  administered  regularly  and  for  a  prolonged  period  gave  me 


NERVOUS  SYMPTOMS  PRODUCED  BY  INTOXICATIONS         599 

very  satisfactory  results.     Hyoscine  hydrobromate  is  also  useful.     Cap- 
sicum associated  with  strychnia  is  commendable. 

For  the  treatment  of  alcoholic  neuritis  see  chapter  on  Multiple  Neuritis. 
In  the  majority  of  cases  of  chronic  alcoholism  institutional  treatment  is 
the  most  efficacious. 

II.  Morphine  Intoxication.    Morphinism 

There  are  two  phases  to  be  considered  in  the  history  of  morphinism. 
One  is  due  to  chronic  intoxication,  the  other  to  abstention  from  the  poison 
after  a  long  period  of  intoxication. 

Symptoms  of  Chronic  Intoxication. — They  usually  appear  after  a  few 
months  of  use  of  the  poison.  The  exact  time  of  their  development  varies 
with  the  individual.     The  symptoms  are  physical  and  mental. 

1 .  The  physical  manifestations  concern  the  motor,  sensory  and  vaso- 
motor apparatuses. 

Motor. — They  are:  muscular  weakness,  tremor,  incoordination,  car- 
diac weakness,  vesical  weakness  and  disturbance  of  ocular  accommoda- 
tion. Epileptiform  convulsions,  attacks  of  angina  pectoris  are  sometimes 
observed. 

Sensory. — They  are:  superficial  or  deep  anaesthesias,  hyperesthesias 
or  paresthesias. 

Vaso-motor. — Diminution  of  secretions.  When  the  salivary  and  seba- 
ceous glands  secrete  insufficiently,  there  is  a  dryness  of  the  mouth  and 
throat,  a  dryness  of  the  skin.  In  the  latter  case  the  skin  is  easily  irritated 
and  furuncles  easily  develop.  The  gastric  and  pancreatic  juices  are  also 
diminished  and  gastro-intestinal  disturbances  are  frequent.  The  men- 
strual flow  is  suppressed.  The  amount  of  urine  is  decreased  and  renal 
congestion  with  albuminuria  are  sometimes  observed.  Impotence  is 
frequent. 

Perspiration  on  the  contrary  is  increased.  A  morphinomaniac  has  a 
pale  face,  cold  extremities,  his  temperature  is  below  normal. 

Cachexia  gradually  develops  and  may  become  alarming,  if  prompt 
treatment  does  not  interfere. 

2.  Psychic  manifestations  are  constant.  The  patient  notices  a  grad- 
ual loss  of  mental  energy.  His  will  power  is  diminished.  His  thoughts 
and  acts  are  characterized  by  an  indifference,  by  apathy.  His  moral 
conceptions  weaken  equally  and  the  tendency  to  deceive  is  marked.  At 
the  same  time  he  is  irritable,  has  outbreaks  of  anger  upon  the  least  pro- 
vocation so  that  at  times  he  may  become  dangerous.  Gradually  the  mem- 
ory becomes  more  and  more  impaired,  the  mental  faculties  low  (dementia). 
Depression  is  marked  and  may  lead  to  suicide.     (See  my  article  in  the 


600        NERVOUS  SYMPTOMS  PRODUCED  BY  INTOXICATIONS 

Jour,  of  Am.  Med.  Ass.,  July,  1908.)  Insomnia  is  very  marked.  Noc- 
turnal hallucinations  of  a  terrifying  nature  torture  the  patient,  but  they 
usually  disappear  during  the  day.  Hallucinations  of  sight  are  more 
frequent  than  any  other  form. 

Symptoms  of  Abstinence. — They  depend  upon  whether  the  suppres- 
sion of  morphine  is  gradual  or  sudden. 

In  case  of  gradual  removal  of  the  drug  there  is  a  certain  general  malaise 
with  loss  of  appetite,  nausea,  vomiting  and  diarrhoea.  Noises  in  the  ear, 
peculiar  visions  are  present.  Neuralgic  pains,  a  general  sensation  of 
cold  distress  the  patient.     He  is  also  in  a  state  of  anxiety  and  depressed. 

When  the  suppression  of  morphine  is  complete  and  sudden,  there  is, 
first  of  all,  a  sense  of  great  muscular  fatigue.  The  perspiration  is  abund- 
ant, the  tremor  is  generalized.  Violent  abdominal  pains  with  vomiting 
and  diarrhoea  are  sometimes  extreme.  A  delirium  with  hallucinations 
develops  rapidly.  In  some  cases  there  may  be  a  cardiac  and  respiratory 
failure  and  death  may  be  imminent.  A  hypodermic  injection  of  morphine 
promptly  administered  may  save  the  patient. 

The  outlook  in  morphinomania  is  unfavorable,  as  recurrences  are 
frequent. 

Treatment- — Withdrawal  of  the  poison  is  the  main  indication.  It 
can  be  accomplished  very  gradually,  or  rapidly  or  else  abruptly.  The 
gradual  method  is  applicable  to  patients  that  are  very  weak  and  more 
or  less  cachectic;  syncope  is  to  be  feared  in  them.  In  such  cases  hypo- 
dermic administration  of  strychnia  fgr.  1/60)  several  times  a  day  is  to  be 
recommended. 

In  robust  individuals  the  rapid  suppression  is  advisable. 

With  the  abrupt  method  one  runs  a  great  risk.  When  cardio-vascular 
disturbance  is  present,  a  sudden  withdrawal  may  bring  on  a  collapse  or 
even  death. 

Whatever  method  is  adapted,  during  the  withdrawal  particular  atten- 
tion should  be  given  to  the  patient's  general  strength.  Alcohol  can  be 
used  freely.  In  extreme  cases  when  collapse  is  threatened,  a  hypo- 
dermic of  morphia  will  improve  the  condition. 

The  gastro-intestinal  disturbances,  which  are  so  conspicuous,  are 
relieved  by  sodium  bicarbonate. 

Good  hygienic  measures,  hydrotherapy  (cold  douches),  massage, 
nutritious  food  are  not  to  be  neglected. 

Finally  the  question  of  prophylaxy  is  the  most  important.  Morphine 
should  be  prescribed  only  in  the  most  urgent  cases  and  a  patient  should 
never  be  trusted  with  a  syringe  and  the  drug.  Institutional  treatment  is 
always  adorable. 


TETANUS  6oi 

Cocaine  Intoxication.     Cocainism 

The  symptoms  of  acute  cocaine  intoxication  are:  general  excitement, 
restlessness,  talkativeness,  paraesthesia  of  the  extremities  and  tinnitus 
aurium.  Sometimes  instead  of  excitation  there  is  on  the  contrary  cere- 
bral depression,  nausea  and  vomiting,  abundant  sweating,  cold  extremities, 
tachycardia.  Epileptiform  convulsions  may  occur.  Recovery  may  fol- 
low, but  death  may  also  take  place  either  in  syncope  or  in  convulsions. 

In  chronic  cocainism  there  is  at  first  a  tendency  to  muscular  and 
mental  agitation.  Gradually  the  latter  becomes  permanent.  A  feeling 
of  exhilaration  in  the  mental  and  sensory  spheres  is  quite  marked.  The 
patient  exhibits  great  activity.  Insomnia  develops.  Hallucinations  make 
then  their  appearance  and  persecutory  delusions  may  accompany  the 
hallucinations.  In  advanced  stages  of  the  disease  the  exaltation  period 
is  replaced  by  a  period  of  depression  and  even  stupor. 

Sensory  phenomena  are  quite  conspicuous.  Various  paraesthesias  are 
present.  Not  infrequently  the  patient  complains  of  a  sensation  of  insects 
crawling  under  his  skin.  Very  often  there  are  sharp  pains  in  the  extrem- 
ities. Anaesthesias  may  be  present.  The  acuity  of  vision  is  diminished. 
Tachycardia,  rapid  respiration,  sweating,  anorexia,  diarrhoea  and  emacia- 
tion are  the  usual  symptoms. 

In  advanced  cases  cachexia  and  mental  enfeeblement  (dementia)  are 
very  marked.  Syncope  may  occur  at  any  time.  (See  my  article  in  the 
Jour,  of  Am.  Med.  Assn.,  July,  1908.) 

Treatment.— S ee  the  preceding  chapter.  It  may  be  added,  however, 
that  sudden  withdrawal  of  cocaine  is  preferable  to  the  slow  method. 


CHAPTER  XXIX 

NERVOUS  SYMPTOMS  CAUSED  BY  SOME  SPECIAL 

INFECTIONS 

TETANUS   (LOCKJAW) 

The  disease  is  due  to  a  special  bacillus  discovered  by  Nicolaier  in 
1885. 

Pathology. — There  are  no  characteristic  changes  in  the  brain  and 
spinal  cord,  but  the  toxins  elaborated  by  the  tetanus  bacillus  reach  the 
central  nervous  system  through  the  nerve  trunks.  Hyperemia,  oedema 
and  minute  hemorrhages  in  the  brain  and  meninges  have  been  observed. 
Congestion  and  softening,  also  changes  in  the  cells  of  the  cord  and  medulla 
have  been  found. 

Symptoms. — A  few  days  after  the  infection  the  muscles  of  the  jaw 
and  the  neck  become  rigid,  producing  the  symptom  trismus,  or  "lockjaw." 
The  patient  is  unable  to  open  the  mouth  freely.  The  masseters  are  very 
hard  to  touch.  The  facies  is  immobile,  forehead  wrinkled,  corners  of  the 
mouth  retracted,  lips  protruded,  eyes  partly  closed.  This  expression  of 
the  face  is  given  the  name  of  "risus  sardonicus."  Gradually  the 
bilateral  tonic  spasm  increases  and  spreads.  The  muscles  of  the 
trunk  and  abdomen  become  involved.  The  patient's  body  is  arched, 
resting  on  his  heels  and  the  back  of  his  head  (opisthotonos).  The  spasm 
of  the  diaphragm  interferes  with  the  breathing.  The  involvement  of  the 
pharyngeal  muscles  produces  difficulty  in  swallowing.  Gradually  the 
muscles  of  the  extremities  become  involved,  although  the  hands  are 
usually  spared.  The  patient  is  confined  to  bed,  his  entire  musculature 
is  rigid,  the  breathing  is  difficult,  the  jaws  are  tightly  pressed  against  each 
other,  the  head  is  drawn  backward.  The  last  touch  or  handling  of  the 
patient  causes  a  convulsive  contraction.  Sometimes  the  latter  occurs 
spontaneously.  It  causes  violent  pain.  Voluntary  urination  and  defeca- 
tion are  impossible.  Constipation  and  retention  of  urine  are  present. 
Sweating  is  a  constant  symptom.  The  temperature  rises  only  before 
death.  The  pulse  is  accelerated.  A  moderate  degree  of  leucocytosis 
has  been  observed  by  Brown  and  others. 

Sensations  are  normal.     The  mentality  is  preserved. 

Course,  Duration,  Prognosis.- — In  the  acute  case  death  may  occur  in 
a  few  days.     Life  is  in  danger  either  from  asphyxia  (laryngeal  spasm), 

602 


TETANUS  603 

cardiac  paralysis,  profound  exhaustion  or  excessive  rise  of  temperature. 
In  subacute  cases  the  outlook  is  favorable.  In  the  acute  forms  the 
mortality  is  high  in  cases  occurring  early  after  severe  trauma.  The 
most  favorable  outlook  is  in  cases  which  develop  late  after  trauma. 

Complications  may  develop  or  follow  tetanus.  Among  them  should 
be  mentioned:  nephritis,  bronchitis  or  bronchopneumonia  from  difficulty 
of  expectoration,  rupture  of  muscles,  fracture  of  the  spinous  processes 
from  violent  spasms.  Sometimes  for  months  after  recovery  a  stiffness  of 
muscles  persists.  Mental  disturbances,  suppuration  of  submaxillary  and 
parotid  glands  have  also  been  observed  after  recovery  from  tetanus. 

Diagnosis. — Strychnine  poisoning  and  hysteria  should  be  always 
thought  of  in  making  a  diagnosis.  In  the  first  the  onset  is  rapid;  gastric 
disturbances  or  tetanic  contractions  of  the  extremities  appear  first, 
while  in  tetanus  trismus  is  first.  Besides,  the  convulsions  are  violent 
and  present  periods  of  complete  relaxation;  the  retina  is  hyperesthetic  and- 
objects  look  green;  duration  is  brief — either  recovery  or  death.  Be- 
sides, a  history  of  poisoning  is  present. 

In  hysteria  there  is  no  trismus.  The  presence  of  hysterical  stigmata 
will  decide  the  diagnosis. 

Hydrophobia  is  recognized  by  absence  of  trismus  and  opisthotonus  by 
violence  of  repiratory  spasms  and  by  psychic  disturbances. 

Etiology. — The  usual  mode  of  infection  with  Nicolaier's  bacillus  is 
through  a  wound.  The  epidemic  or  sporadic  character  of  tetanus  is  well 
known.  It  is  more  prevalent  in  the  hot  than  in  the  cold  months  of  the 
year. 

Infection  may  also  take  place  in  new-born  children  through  the  um- 
bilicus. This  is  tetanus  neonatorum.  Trismus  is  here  the  most  con- 
spicuous symptom,  which  prevents  the  infant  from  taking  nourishment. 
The  prognosis  is  very  grave. 

Treatment. — Prophylaxis,  consisting  of  rigorous  antiseptic  measures, 
is  urgent  in  every  case  of  infection. 

As  soon  as  the  wound  occurs,  thorough  disinfection  and  thorough 
cauterization  of  it  must  be  done  as  promptly  as  possible.  Immediately 
or  very  shortly  after,  antitoxin  as  a  preventive  measure  should  be  used. 
About  500  units  should  be  injected  for  this  purpose.  The  early  adminis- 
tration of  antitoxic  serum  is  of  great  importance  and  Behring  urges  its 
administration  within  the  first  twenty-four  hours.  Unless  it  is  injected 
before  trismus  makes  its  appearance,  its  value  is  very  small.  Vaillard 
{Bull,  de  VAcad.  de.  Med.,  1908)  injected  the  serum  as  a  prophylactic 
measure  in  13,124  horses  and  none  contracted  tetanus.  His  observations 
also  show  that  when  tetanus  developed  notwithstanding  the  preventive 


604  TETANUS 

injections,  its  course  was  milder.  The  high  mortality  depends  largely 
upon  the  late  use  of  the  serum.  As  to  the  amount  to  be  injected,  it  is 
advisable  to  inject  at  first  2.25  grm.  and  0.5  grm.  for  each  of  the  following 
doses.  In  extreme  cases  instead  of  subcutaneous  injections  the  serum 
may  be  injected  into  the  spinal  canal  after  a  small  amount  of  cerebro- 
spinal fluid  is  allowed  to  escape. 

Although  satisfactory  results  have  been  reported,  nevertheless  a  great 
many  failures  have  been  observed. 

General  measures  should  not  be  neglected.  Absolute  quiet,  avoidance 
of  manipulation  of  the  body  are  necessary.  Feeding  should  be  done 
through  the  nasal  cavities,  if  trismus  exists.  Bromides,  chloral,  morphia, 
warm  baths  will  be  utilized  for  relief  of  the  tonic  spasms.  In  spite  of  the 
spasms,  cardiac  stimulants  are  sometimes  necessary.  Alcohol,  strychnine, 
digitalis,  camphor  are  the  most  useful  drugs.  The  latter  three  are 
to  be  given  hypodermically.  Care  must  be  taken  of  the  functions  of  the 
viscera. 

In  1905  Meltzer  proved  experimentally  that  magnesium  possesses  an 
inhibiting  power  over  the  processes  of  the  body,  viz.  inhibits  respiration 
and  produces  paralysis  when  given  by  intravenous  injections;  when  in- 
jected subcutaneously,  it  produces  narcosis  with  muscular  relaxation; 
when  injected  into  the  spinal  canal  it  produces  anesthesia.  Since  then 
it  has  been  shown  that  an  intraspinal  injection  of  magnesium  sulphate 
in  cases  of  tetanus  controls  the  convulsions  and  leads  to  recovery. 
The  quantity  to  be  injected  is  1  c.c.  of  25  per  cent,  solution  to  each  25 
pounds  of  body  weight.  The  injections  may  be  repeated  several  times 
in  case  the  results  of  the  first  injections  are  nol  entirely  satisfactory. 
Kocher  (Corresp.-Blatt  f.  Schweiz.  Aerzte.,  1912)  believes  that  15  per 
cent,  or  even  10  per  cent,  solution  of  magnesium  sulphate  is  sufficient. 
The  greater  susceptibility  of  children  to  toxic  action  must  be  borne  in 
mind:  2  c.c.  should  be  the  maximum  dose  for  a  child.  In  view  of  the 
cumulative  action  of  the  drug,  repetition  of  the  dose  should  be  made 
not  oftener  than  at  an  interval  of  twenty-four  hours.  Kocher  warns 
that  if  no  cerebro-spinal  fluid  flows  upon  lumbar  puncture,  injection  of 
magnesium  sulphate  is  dangerous.  The  injections  of  magnesium  may 
be  associated  with  administration  of  antitetanic  serum.  This  latter  com- 
bined method  has  given  highly  favorable  results. 

Baccelli  treats  tetanus  with  injections  of  carbolic  acid  in  2  per  cent, 
solution.  He  commences  with  a  daily  dose  of  0.30  to  0.50  watching  care- 
fully the  condition  of  the  urine  and  the  patient's  tolerance.  Gradually 
he  increases  the  dose  to  1  grm.  in  several  injections  in  twenty-four  hours. 
In  very  grave  cases  higher  doses  may  be  given.     Baccelli  claims  (Berl. 


HYDROPHOBIA  605 

klin.  Wchn.,  191 1)  that  with  his  method  the  mortality  in  his  ordinary  cases 
has  been  only  2.12  per  cent,  and  in  the  very  grave  cases  18.5  per  cent. 

Atkey  (Lancet,  1913)  basing  himself  on  the  effects  produced  by 
Souttar  by  injecting  paraldehyde  and  ether  intravenously,  used  these 
drugs  in  conjunction  with  copious  injections  of  normal  saline  solution  in 
treatment  of  tetanus.  In  a  case  of  a  boy  of  19,  he  made  twelve  intravenous 
injections  of  a  solution  of  paraldehyde  and  ether,  5  c.c.  each  in  150  c.c. 
of  normal  solution  and  the  result  was  excellent.  The  boy  recovered. 
The  drugs  apparently  act  as  an  anaesthetic  and  hypnotic. 

Under  the  name  of  Cephalic  Tetanus  is  described  a  variety  of  tetanus 
which  is  characterized  by  spasms  confined  mainly  to  the  head  and  face  and 
which  results  from  injuries  of  the  head.  Spasm  of  the  facial  muscles,  then 
trismus,  spasms  of  pharyngeal,  laryngeal  and  respiratory  muscles  are  the 
symptoms  observed.     The  rigidity  may  spread  to  other  muscles. 

This  form  is  also  called  hydrophobic  tetanus,  because  of  the  spasmodic 
contractions  of  the  oesophagus.     The  majority  of  cases  ends  fatally. 

Hydrophobia.     Rabies.    Lyssa 

The  infectious  element  of  this  disease  is  not  yet  known.  It  attacks 
animals  and  from  them  is  communicated  to  man  by  inoculation  through 
a  bite.     The  exact  nature  of  the  virus  is  unknown. 

Pathology. — In  1900  Van  Gehuchten  and  Nelis  discovered  what  they 
believed  to  be  of  diagnostic  value,  changes  in  the  intervertebral  ganglia, 
consisting  of  proliferation  of  cells,  probably  from  the  capsules  of  the 
ganglia.  The  ganglionic  cells  themselves  degenerate,  inasmuch  as  the  pro- 
toplasma  with  the  nucleus  in  some  cases  totally  disappears  and  is  re- 
placed by  the  newly  formed  small  cells.  These  changes  were  found 
not  only  in  the  spinal,  but  also  in  the  pneumogastric  and  Gasserian 
ganglions.  Babes  also  described  a  so-called  "rabic  tubercle,"  consisting 
of  an  accumulation  of  embryonal  cells  around  the  vessels  and  nerve  cells 
of  the  anterior  cornua  of  the  cord  and  in  the  medulla.  The  cells  of  the 
latter  undergo  degeneration,  viz.  chromatolysis  with  disappearance  of  the 
nuclei;  the  pericellular  spaces  are  dilated  and  filled  with  the  characteristic 
embryonal  cells.  I  have  seen  the  same  "rabid  tubercles"  around  the 
pyramidal  cells  of  the  cortex  in  several  cases  of  rabies.  In  1903  Negri's 
bodies  became  a  new  element  in  the  diagnosis  of  rabies.  They  are  found 
in  the  nerve  cells  and  in  their  largest  dendritic  prolongations  especially  of 
the  cornu  ammonis  but  also  of  the  cortex,  pons,  medulla  and  spinal 
ganglia. 

These  bodies  are  of  1  to  27  ,«  in  size,  round  or  oval;  they  are  surrounded 
by    a    hyaline   membrane.     They   are   very  resistant  and  may  be  pre- 


6o6 


HYDROPHOBIA 


served  for  many  days  when  the  tissue  is  preserved  in  glycerine.     The 
method  for  bringing  them  out  to  the  best  advantage  is  as  follows: 

Impregnate  the  tissue  in  a  solution  with  a  sublimate  base,  especially  a 
sublimate  with  acetic  alcohol  of  Gilson  or  sublimate  of  von  Lenhossek. 
Next  step  is  to  cover  the  piece  with  a  slight  layer  of  thick  celloidine,  dry 
in  the  air  for  a  few  minutes,  place  tissue  in:  alcohol  absolute  50  c.c.+ 
chloroform  5  c.c.  Avoid  xylol  for  clearing,  but  use  toluidin  or  cedar  oil. 
It  is  admitted  now  that  Negri's  bodies  are  pathognomonic  of  rabies.  As  to 
their  nature,  Negri  believes  them  to  be  parasites  undergoing  degeneration. 


Fig.  169. — Rabies.     Spinal  Ganglion.     {Original.) 

Symptoms. — After  a  period  of  incubation,  which  may  last  from  a 
few  days  to  two  months  (in  children  shorter  than  in  adults),  the  infected 
part  produced  by  the  bite  of  the  animal  begins  to  itch  and  burn;  some- 
times there  is  pain,  lancinating  in  character.  Headache,  insomnia  and 
anorexia  develop  next.  The  patient  becomes  depressed  and  irritable. 
A  state  of  anxiety  appears  and  this  is  increased  by  a  rigidity  of  the  muscles 
of  the  throat  and  difficulty  in  swallowing.  At  the  same  time  the  tempera- 
ture rises  slightly,  the  pulse  is  accelerated. 

Soon  another  phase  of  the  disease  makes  its  appearance.  It  is  char- 
acterized by  restlessness,  cerebral  excitement  and  hyperaesthesia.  The 
least  noise,  a  bright  light,  a  slight  breeze  causes  a  spasm.  The  latter 
affects  especially  the  muscles  of  the  larynx  and  pharynx.  As  there  is 
intense  thirst,  the  patient  makes  great  efforts  to  drink,  but  an  attempt  to 


HYDROPHOBIA  607 

take  water  or  the  sight  of  water  or  even  the  suggestion  of  it  produces  a  pain- 
ful spasm  of  the  pharynx  and  larynx  (hydrophobic  spasm).  Dyspncea  is 
then  pronounced.  The  fear  of  water  (hydrophobia)  is  characteristic  of 
the  disease.  The  difficulty  of  swallowing  makes  the  saliva  dribble  from 
the  mouth.  The  temperature  goes  up  to  ioi°-io3°.  At  the  same  time 
a  delirium  with  hallucinations  and  delusions  develop.  The  convulsive  at- 
tacks which  were  at  first  confined  to  the  muscles  of  deglutition  and 
respiration,  now  become  generalized  and  may  be  so  frequent  that  asphyxia 
occurs.  They  are  brought  on  by  the  same  excitants  which  produce 
the  spasm  of  pharynx  and  larynx.  During  the  convulsions  patients 
make  noises  resembling  the  barking  of  a  dog.  The  delirium  is  usually 
marked  during  the  paroxysms;  the  patient  may  become  violent,  inflict 
injuries  on  himself  or  bite  others  and  spit  at  them.  This  phase  of  the 
disease  usually  lasts  one  or  three  days.  This  may  be  followed  by  a  para- 
lytic stage,  during  which  all  the  previous  symptoms  gradually  abate  and  the 
patient  becomes  quiet.     Gradually  he  sinks  into  unconsciousness  and  dies. 

Paralytic  Rabies.— In  exceptional  cases  the  convulsive  phase  is  absent 
and  there  is  only  the  paralytic  phase.  It  is  a  very  grave  condition.  It 
usually  follows  very  extensive  bites.  Paralysis  rapidly  develops  and 
assumes  the  type  of  acute  ascending  paralysis.  Death  follows  from 
respiratory  or  cardiac  paralysis. 

Course,  Duration,  Prognosis. — The  rapidity  of  development  of  the 
symptoms  depends  upon  the  part  of  the  body  bitten.  Wounds  on  the 
face  present  the  greatest  danger.  The  prognosis  is  grave,  except  when 
antitoxic  treatment  is  early  instituted.  Death  usually  occurs  within 
two  or  five  days. 

Diagnosis. — -In  tetanus  there  is  also  a  spasm  of  deglutition  and  of 
respiratory  muscles,  but  the  presence  of  trismus  and  absence  of  fear  of 
water  will  decide  the  diagnosis. 

Hysterical  paroxysms  may  sometimes  simulate  rabies  (pseudo-hydro- 
phobia), but  the  special  symptoms  of  hysteria  will  soon  determine  the 
disease. 

Treatment. — As  soon  as  the  bite  occurs,  a  ligature  is  placed  above  the 
wound  and  kept  in  place  several  hours.  The  latter  should  be  enlarged 
and  a  thorough  cauterization,  followed  by  an  antiseptic  dressing,  be  done. 
It  should  be  left  open  and  free  drainage  given  for  several  weeks. 

As  soon  as  possible  the  antirabic  serum  should  be  employed.  Since 
Pasteur  introduced  this  treatment,  the  mortality  has  greatly  decreased. 

The  other  symptoms  of  the  disease  are  treated  accordingly.  Perfect 
quiet,  sedatives  (bromides,  chloral,  chloroform,  morphia),  rectal  feeding, 
if  not  possible  by  the  mouth,  are  practically  all  that  can  be  done. 


608  HYDROPHOBIA 

NERVOUS  MANIFESTATIONS  IN  PELLAGRA 

The  nature  of  the  disease  is  still  unsettled.  The  old  view  with  relation 
of  the  disease  to  ingestion  of  ma'ize  is  not  held  by  all  and  considered  as 
doubtful.  Sambons  idea  in  regard  to  an  infectious  origin  of  the  disease 
is  viewed  with  favor.  The  endemic  mode  of  propagation,  its  appearance 
at  certain  seasons,  the  failure  of  all  prophylactic  measures  based  on  the 
theory  of  ma'ize,  all  speak  in  favor  of  infection.  It  is  supposed  that 
pellagra  is  an  insect-borne  disease. 

The  manifestations  of  pellagra  concern  the  gastro -intestinal  tract, 
skin  and  the  nervous  system. 

The  affection  begins  usually  with  gastro-intestinal  and  psychic  dis- 
turbances. Gastritis  and  diarrhoea  are  frequent  in  the  early  stages.  The 
patient  is  depressed,  indifferent  to  surroundings  and  to  his  own  appearance, 
or  else  may  have  maniacal  outbursts.  He  is  forgetful,  commits  errors 
of  serious  nature.  Sudden  lapses  of  memory  resembling  Petit  Mai, 
vertigo  are  not  infrequent.  Exceptionally  delusions  and  hallucinations 
are  observed.  Suicidal  tendencies  are  not  rare.  Paresthesias,  headache, 
pain  in  back  or  in  the  extremities  of  tabetic  nature,  hypaesthesias  or 
anaesthesias,  tremor  of  the  hands  and  tongue  resembling  that  of  multiple 
sclerosis,  muscular  spasm,  Romberg's  sign,  increased  patellar  tendon  re- 
flexes, occasionally  Babinski's  sign — are  symptoms  which  may  all  or  only 
some  of  them  be  encountered  in  Pellagra. 

Eye  Symptoms. — Early  forming  cataracts  are  frequent.  Retinitis  and 
optic  neuritis  are  not  uncommon.  The  choroid  is  quite  frequently 
involved. 

The  skin  manifestations  consist  of  symmetrical  in  shape  and  position 
erythemata  resembling  sunburn;  they  are  seen  on  the  exposed  parts  of 
the  body,  especially  on  the  hands.  Desquamation  is  the  ultimate  result. 
Changes  in  the  postero-lateral  tracts  of  the  spinal  cord  have  been  found  in 
some  cases. 

Treatment. — Mild  cases  require  no  treatment.  In  cases  with  severe 
gastro-intestinal  disorders  and  asthenia,  arsenic  is  indicated.  Nutri- 
tious diet  with  rest  are  the  chief  measures  to  be  relied  upon.  The 
mortality  is  quite  high. 


INDEX 


Abasia,  468 
Abducens  nerve,  401 
Abscess  of  brain,  106 

course,  109 

diagnosis,  no 

etiology,  106 

pathology,  107 

prognosis,  no 

symptoms,  108 

treatment,  in 
Abscess  of  cerebellum,  218 
Abstinence,  600 
Abulia,  458 

Achilles  tendon  reflex,  247 
Achondroplasia,  576 
Acromegaly,  572 
Acroparsesthesia,  583 
Acustic,  striae,  15 

tubercle,  15 
Acute  ascending  paralysis,  396 
Adiadochokinesia,  215 
Adiposis  dolorosa,  577 
Adipositas,  154 
Affect,  479 
Agenesis,  117 
Agoraphobia,  457 
Agraphia,  78,  131,  149 
Akinesia  algera,  533 
Ala  cinerea,  15 
Albumen,  371 
Alcoholic  injections,  433,  436 

intoxication,  595 

neuritis,  387 
Alexia,  78,  131,  133,  150 
Allochiria,  69 
Alternating  ptosis,  402 
Amaurosis,  467 
Amaurotic  form  of  tabes,  250 
Ammonis  cornu,  81 
Amnesia,  153,  367,  469,  484,  550 

verbal,  135 
Amusia,  132 

Amyotonia  congenita,  327 
Amyotrophic  lateral  sclerosis,  319 
Anaesthesia,  68,  245,  292,  309,  333,  387,  391, 

<  417,  4i8,  427,  465 
Analgesia,  245 
Anarthria,  227 
Anemia  of  brain,  202 
Anencephalia,  52 
Aneurism,  87,  144 
Angioma,  144 
Angioneuroses,  560 
Angioneurotic  oedema,  584 
Angular  gyrus,  131 
Ankle-clonus,  71,  90 
Anosmia,  399 
Anterior  commissure,  3 

cornua,  3 

crural  nerve,  425 

poliomyelitis  (acute),  264 

39 


Anxiety,  457 

neurosis,  464 
Aphasia,  78,  96,  130,  149,  469 

of  conduction,  133 

congenital,  135 

motor,  130 

optic,  135 

sensory,  132 

transcortical  or  pure,  134 
Aphemia,  78,  130 
Aphonia,  228 
Apoplexy,  82 

delayed,  101 

ingravescent,  89,  101 

spinal,  280 
Apraxia,  138,  154 
Aqueduct  of  Sylvius,  21,  163 
Arachnoid,  of  brain,  45 

of  spinal  cord,  9 
Aran-Duchenne,  314 
Argyll-Robertson  pupil,  246,  402 
Arhinencephalia,  55 
Arsenical  intoxication,  592 

paralysis,  390 

preparations,  253 
Arteries,  anterior  cerebral,  46 

basal,  48 

internal  carotid,  46 

middle  cerebral,  48 

posterior  cerebral,  48 

vertebral,  48 
Arthritic  muscular  atrophy,  327 
Arthropathies,  247,  289 
Ascending  neuritis,  387 

paralysis,  396 
Asphyxia  (local),  587 
Association  area,  79 

method,  463 
Astasia-abasia,  60,  468 
Astereognosis,  69,  79,  95,  150 
Asthenia,  215 

Asthenic  bulbar  paralysis,  231 
Asymbolia,  69 
Ataxia,  60,  167,  213,  220,  242,  244 

Friedreich's,  261 
Ataxic  paraplegia,  259 
Athetosis,  61,  116,  167,  262,  503 
Atonia,  327 
Atremia,  534 
Atrophy,  62,  116,  267,  288,  332 

arthritic,  327 

dystrophy,  321 

of  brain,  114 

of  muscles,  314,  417 

primary  neuritic,  325 

progressive  of  infants,  318 

progressive    of     spinal    origin, 

3i4 
Auditory  apparatus,  20 

nerve,  411 
Aura,  470,  482 

609 


6io 


INDEX 


Automatism,  469 
Autosuggestion,  469 

Babinski,  73,  91,  479 

Bar  any,  404 

Basal  ganglia,  27,  152,  167 

meningitis,  340 
Base  of  brain,  151 
Basedow's  disease,  560 
Bell's  palsy,  406 
Benedict's  syndrome,  239 
Beriberi,  392 
Betz  cells,  76 
Birth  palsy,  417 
Blepharospasm,  467,  515 
Brachial  neuralgia,  437 

plexus,  416 
Brachium,  anterior,  20 

posterior,  20 
Breuer,  463 
Brissaud,  569 

Broca's  aphasia,  130,  131,  192 
Brown-Sequard's  paralysis,  283 
Brudzinski's  sign,  177 
Burdach's  columns,  6,  243 

nucleus,  14 

Cachexia  strumipriva,  567 
Caisson  disease,  284 
Calamus  scriptorius,  15 
Cancer  of  vertebras,  311 
Capsule,  external,  30 

internal,  31 
Carbonic  gas  intoxication,  593 

neuritis,  391 
Catalepsy,  470 

cerebellar,  215 
Cauda  equina,  10,  293 
Caudate  nucleus,  27 
Centers,  75 

auditory,  78,  80 

gustatory,  80 

intelligence,  81 

motor,  75 

olfactory,  80 

sensori-motor,  76 

sensory,  79 

speech,  78 

stereognostic,  79 

visual,  80 

writing,  78 
Central  canal,  3 
Cephalalgia,  535 
Cerebellar  abscess,  218 
asynergy,  214 
ataxia,  213,  262 
catalepsy,  215 
heredo-ataxia,  219 

peduncles,  19 

posterior  inferior  artery,  235 
Cerebellum,  40,  212 
Cerebral  hemispheres,  26 

hemorrhage,  82 

localization,  75 
Cerebritis,  104 
Cerebro-spinal  fluid,  371 


Cervical  enlargement,  1 

nerves,  416 

pachymeningitis  (hypertrophic),  331 
Cervico-occipital  neuralgia,  436 
Charcot,  288,  319 
Choked  disc,  147,  215,  400 
Choleastoma,  145 
Chorea,  61,  116,  167,  262,  495 

chronic,  510 

hereditary,  501 

Huntington's,  501 

electric,  519 

Dubini's,  519 
Choroid  plexus,  15,  46 

tela,  15 
Chronic  chorea,  501 
Chwostek's  sign,  521 
Cingulum,  37 
Circle  of  Willis,  48 
Circumflex  nerve,  419 

Circumscribed  serous  spinal  meningitis,  333 
Cisterna  magna,  184 
Clarke's  column,  6,  288 
Claudication,  334 
Claustrophobia,  457 
Clavus  hystericus,  466 
Claw-like  hand,  288,  315,  422,  428 
Cocaine  intoxication,  601 
Coccygodynia,  446 
Cochlear  nerve,  411 
Columns,  6 

of  Burdach,  6 

of  Goll,  6 
Combined  sclerosis,  259 
Commissural  fibers,  37 
Commissure,  3 

anterior,  38 
Compression  of  medulla,  235 

of  cord,  303 

hippocampal,  38 
Concussion  of  brain,  209 

of  cord,  302 
Conjugate  deviation,  89 

movement,  402 
Contraction  of  visual  fields,  467 
Contracture  (secondary),  92,  153 
Contusion  of  cord,  302 
Conus  medullaris,  1,  291 
Conversion,  479 

Convulsions,  147,  150,  151,  163,  176,  192,  483 
Coprolalia,  508 
Cord,  1 
Cornua,  3 

anterior,  3 

lateral,  6 

posterior,  6 
Corona  radiata,  31 
Corpora  quadrigemina,  169 

striata,  27,  168 
Corpus  callosum,  31,  152,  153 
Coxalgia,  441,  468 
Craniorrhachischisis,  57 
Cretinism,  570 
Crisis,  245,  249 
Crossed  pyramidal  tract,  8 

paralysis,  234,  237,  239 


INDEX 


6ll 


Crura,  19,  20 

Crural  (anterior)  nerve,  425 

neuralgia,  441 
Crutch  palsy,  421 
Cushing,  121 
Cyclopia,  55 
Cycloplegia,  402 
Cysticercus,  145 
Cysts,  145,  158,  162,  306 

Dancer's  cramp,  528 
Deafness,  411 

Decompression  (cerebral),  160 
Decubitus,  277 
Decussation,  8 

of  pyramids,  1 1 
Defense-neurosis,  479 
Deficient  will,  458 
Degeneration,  ascending,  242 

descending,  85 
Dejerine,  334,  383 
Delire  du  toucher,  457 
Delirium,  108,  176,  192,  210,  471 

tremens,  595 
Dementia,  153,  258,  357,  484,  601 

paralytica,  353 
Dendrites,  49 
Dentate  ligaments,  10 

nucleus,  40 
Diaphragmatic  phenomenon,  41 
Diphtheritic  paralysis,  390 
Diplegia,  61,  117 
Diplomyelia,  58 
Diplopia,  246,  401 
Direct  cerebellar  tract,  8 

pyramidal  tract,  8 
Disseminated  sclerosis,  295 

diagnosis,  300 

forms,  299 

pathology,  295 

prognosis,  300 

symptoms,  296 

treatment,  301 
Divers'  paralysis,  284 
Double  personality,.  469 
Doubts,  457 
Dreamy  state,  482 
Dubini's  chorea,  519 
Dura  mater,  of  brain,  43 

of  spinal  cord,  9 
Dysarthria,  227 

Dysbasia  lordotica  progressiva,  505 
Dyschromatopsia,  147 
Dyskinesia  algera,  533 
Dysopsia  algera,  534 
Dystonia  musculorum  deformans,  505 
Dwarfishness,  576 

Early  paresis,  366 
Echinococcus,  145,  158 
Echokinesis,  511 
Echolalia,  131,  511 
Ehrlich,  253 

Electrical  contractility,  63,  270 
Electric  chorea,  519 
Elephantiasis,  574 


Embolism,  86 
Encephalitis,  104,  354 
acute,  104 
chronic,  112 
congenital,  115 
non-suppurative,  104 
suppurative,  106 
Encephalocele,  55 
Encephalopathy,  363,  591 
Endothelioma,  144 
Epiconus,  295 
Epicritic,  70 
Epidural  injections,  443 
Epilepsy,  117,  482 

equivalents,  487 

major  (Grand  Mai),  482 

minor  (Petit  Mai),  485 

psychic,  486 

reflex,  491 

senile,  490 
partialis  continua,  128 
Jacksonian  or  focal,  124 

diagnosis,  126 

etiology,  127 

pathology,  124 

prognosis,  126 

symptoms,  124 

treatment,  128 
Epileptic  character,  487 
Epiphysis,  26 
Epithalamus,  26 
Equilibration,  545 
Equino-varus,  427 
Erb,  231,  309,  323,  346 
Erb's  paralysis,  417 

sign,  521 
Erythromelalgia,  585 
Exophthalmic  goiter,  560 
Exsner,  255 
External  cutaneous  nerve,  426 

Fabrications,  388 
Facial  hemiatrophy,  581 

hemihypertrophy,  583 

hemispasm,  467 

nerve,  406 

neuralgia,  435 

paralysis,  406 

spasm,  514 
Falx  cerebelli,  44 

cerebri,  44 
Family  spastic  paralysis,  257 
Faradism,  63 
Fatigue,  455 
Festination,  531 
Fibers,  ascending,  32 

association,  37 

commissural,  37 

descending,  35 

frontal,  36 

inferior  longitudinal,  37 

internal  arcuate   17 

motor,  35,  49 

occipital,  35 

occipito-frontal,  37 

sensory,  35,  49 


6l2 


INDEX 


Fibers,  superior  longitudinal,  37 

temporal,  36 

uncinate,  37 
Fibril  ary  contractions,  316,  325,  51S 
Fillet,  16 

Filum  terminale,  1 
Fissures,.  3 

longitudinal,  26 
Fixed  idea,  478 
Flechsig's  tract,  8 
Flexner,  194,  265 
Focal  epilepsy,  1 24 
Folie  de  doute,  457 
Foot-drop,  386,  391,  427 
Fornix,  40 

Forster's  operation,  122,  254 
Four  reactions,  374 
Fourth  ventricle,  15 
Fovea,  15 

Fracture  of  skull,  205 
Franke,  254 

Free  association  method,  463 
Frenkel,  255 
Freud,  463 

Friedreich's  ataxia,  220,  261 
Frohlich's  syndrome,  154 
Funiculus  teres,  15 


Galvanism,  65 
Ganglia,  basal,  27,  167 

Gasserian,  434,  436 

spinal,  243 
Gas  poisoning,  169 
Gastric  crises,  249 

tetany,  523 
Gelatinous  substance  (Rolando),  6 
General  paralysis  of  the  insane,  353 
Geniculate  bodies,  20,  25 
Gigantism,  575 
Gilles  de  la  Tourette,  508 
Gill's  operation,  100,  121 
Girdle  pain,  245,  274 
Glioma,  143,  158 
Globus  hystericus,  467 

pallidus,  30 
Glosso-pharyngeal  nerve,  412 
Gluteal  nerves,  426 
Goll's  columns,  6,  243 

nucleus,  13 
Gordon,  73,  91,  93,  150 
Grand  Mai,  482 
Graves'  disease,  560 
Gray  substance,  3 
Gumma,  144,  340 

Haines'  operation,  184 
Hallucinations,  359 
Head,  70,  447 
Headache,  146,  339,  451,  535 

indurative,  538 
Hearing,  70 
Hematoma,  171,  206 

of  the  ear,  358 
Hematomyelia,  279 
Hemianesthesia,  94,  98,  150,  167,  465 


Hemianopsia,  80,  140 
Hemiasynergy,  214 
Hemiataxia,  167 
Hemiatrophy,  415,  581 
Hemichorea,  497 
Hemicrania,  539 
Hemihypertrophy,  583 
Hemiplegia,  61,  90,  167 

crossed,  234 

infantile,  112 

syphilitic,  341 
Hemispasm,  facial,  467 
Hemorrhage,  cerebral,  82,  97 
diagnosis,  96 
etiology,  85 
localization,  98 
pathology,  82 
prognosis,  96 
symptoms,  88 
treatment,  99 
Hemorrhage  of  cerebellum,  221 

medulla,  234 

meningeal,  100,  113,  333 

of  pons,  237 

in  spinal  cord,  279 
Hereditary  chorea,  501 

syphilis,  349 
Heredo-ataxia  (cerebellar),  58 
Herpes  zoster,  436,  447 
Heterotopia,  58 
High  steppage  gait,  60 
Hippocampal  commissure,  38 
Hippocampus,  81 
Hippus,  405 
Hoche's  bundle,  8 
Hoffmann's  sign,  521 
Hoover's  phenomenon,  475 
Horsley,  161 

Horsley's  operation  for  epilepsy,  128 
Hunt,  R.,  407,  424 
Huntington's  chorea,  501 
Hydatid  cysts,  306 
Hydrocephaloid  anemia,  202 
Hydrocephalus,  114,  162 

acute,  162 

chronic,  162 

diagnosis,  164 

etiology,  165 

external  162 

internal,  114 

pathology,  162 

symptoms,  163 

treatment,  165 
Hydromyelia    286 
Hydrophobia,  605 
Hypaesthesia,  68,  465 
Hypersesthesia,  68,  $33,  387,  466 
Hyperalgesia,  246 
Hyperemia,  203 
Hpyerplasia,  54 

Hypertrophic  cervical  pachymeningitis,  331 
Hypertrophy,  62 

of  brain,  115 
Hypnotism,  477,  478,  481 
Hypochondria,  458 
Hypoglossus  nerve,  415 


INDEX 


613 


Hypophysis,  26 
Hypoplasia,  52 
Hypothalamic  nucleus,  22 
Hypotonia,  61,  215,  244,  506 
Hysteria,  464 
Hysterical  hemiplegia,  475 

insanities,  472 

paroxysms,  470 
Hysteroepilepsy,  472 
Hysterogenetic  zones,  466 
Hysteroid,  485 

Idiot,  569 

Incipient  tabes,  251 
Indurative  headache,  538 
Infantile  hemiplegia,  112,  115 

spinal  paralysis,  264 
Infantilism,  569,  576 
Ingravescent  apoplexy,  89,  101 
Insomnia,  148,  452 
Insular  sclerosis,  295 
Intention  tremor,  262,  297 
Intercostal  neuralgia,  438 

zona,  448 
Intermittent  claudication,  445 
of  spinal  cord,  334 

closing  of  cerebral  arteries,  102 
Internal  capsule,  31,  99 
Intracranial  pressure,  145 
Iridoplegia,  402 

Jacksonian  epilepsy,  124 
Janet,  478, 
Jargonaphasia,  132 
Jendrassik's  sign,  247 
Juvenile  paresis,  261 
tabes,  256 

Kakke,  392 

Keratitis,  neuroparalytic,  405 

Kernig's  sign,  176,  192,  330 

Kleptomania,  458 

Klumpke,  310,  418 

Knee-jerk,  72 

Kocher's  operation  for  epilepsy,  128 

Kojewnikoff,  128 

Korsakoff,  210,  388 

Krause,  166,  218 

Laceration  of  cord,  303 

Lacunes,  83,  258 

Laminectomy,  312,  333 

Lancinating  pain,  245 

Landouzy-Dejerine  type  of  muscular  atrophy, 

323 
Landry's  paralysis,  396 
Larnygeal  nerve,  413 
Lateropulsion,  60,  531 
Lead  intoxication,  363,  591 

palsy,  389 
Lemniscus,  16 

lateral,  17,  21 

median,  17,  21 
Lenticular  degeneration,  168 

nucleus,  29 

zone,  137 


Lepra  neuritis,  393 

Lethargy,  470 

Letter  blindness,  133 

Leucocytosis,  372 

Liepman,  138 

Ligula,  15 

Limbic  lobe,  81 

Lissauer's  tract,  7 

Little's  disease,  112,  117,  257 

diagnosis,  120 

pathology,  117 

prognosis,  120 

symptoms,  119 

treatment,  120 
Local  asphyxia,  588 

syncope,  588 
Lockjaw,  602 
Locomotor  ataxia,  242 
Locus  niger,  21 
Long  thoracic  nerve,  418 
Longitudinal  fissure,  26 
Lumbar  enlargement,  1,  310 

neuralgia,  444 

puncture,  128,  160,  166,  182,  208,    369, 

375 
Lumbo-abdominal  neuralgia,  444 

-sacral  nerves,  425 
Luys  body,  22 

Lymphocytosis,  189,  358,  374 
Lymphorrhagia,  231 
Lyssa,  605 

Macrocephaly,  55,  576 
Magnesium  sulphate,  604 
Main  en  griffe,  288,  315 
Malformations,  51 
Mai  perforant,  248 
Mammillary  bodies,  40 
Manganese  intoxication,  594 
Marantic  thrombosis,  199 
Marie,  P.,  78,  136,  219 
Mastodynia,  438 
Median  nerve,  424 
Medico-legal  considerations,  551 
Medulla  oblongata,  11,  234 

compression  of,  235 
Meniere's  disease,  545 
Meningeal  hemorrhage,  100,  113 
Meninges,  of  brain,  43 

of  spinal  cord,  9 
Meningitis,  171 

leptomeningitis,  173 
acute,  173        "B 

non-tubercular,  173 

circumscribed,  178,  333 

gonoccal,  180 

grippal,  179 

in  lcoholics,  181 

in  old  age,  181 

otitic,  180 

pneumococcus,  179 

serous,  178,  597 

syphilitic,  180,  340 

traumatic,  179 

typhoid,  179 

tubercular,  185 
aseptic  cerebro-spinal,  198 


614 


INDEX 


Meningitis,  leptomeningitis,  aseptic  cerebro- 
spinal basal,  340 

basic,  193 

chronic,  197 

epidemic,  190 

spinal,  269,  329 
Meningismus,  178,  182 

pachymeningitis,  171,  331,  344 
Meningocele,  55 
Meningococcus,  174,  372 
Meningoencephalitis,  250,  338 
Meningomyelitis,  344,  345,  346 
Meningomyelocele,  56 
Mental  disturbances,  148,  153,  210,  220,  341, 

348,  356,  359,  366,  388,  562,  595,  599 
Meralgia  paraesthetica,  444 
Mercurial  neuritis,  392,  593 
Metallic  poisons,  591 
Metatarsalgia,  447 
Microcephalia,  53 
Microgyria,  53,  115 
Micromyelia,  576 
Midbrain,  20 
Migraine,  539 

Millard-Gubler's  syndrome,  239 
Milroy's  disease,  585 
Mirror  writing,  131 
Mobius'  sign,  561 
Monophobia,  457 
Monroe  foramen,  38,  163 
Moria,  148 
Morphea,  579 
Morphine  intoxication,  599 
Morvan's  disease,  290 
Motor  area,  75,  149 

pathway,  8 

phenomena,  59 
Multiple  neuritis,  385 

alcoholic,  387 

arsenical,  390 

beriberi,  392 

carbonic  gas,  391 

diphtheritic,  300 

lead,  389 

lepra,  393 

mercurial,  392 

puerperal,  392 

senile,  394 
Multiple  sclerosis,  295 
Musculocutaneous  nerve,  420 

-spiral  nerve,  420 
Myasthenia  gravis   231 
Myasthenic  reaction,  232 
Myatonia  congenita,  327 
Myelitis,  272 

acute,  272 

diagnosis,  276 
etiology,  273 
pathology,  272 
prognosis,  275 
symptoms,  273 
treatment,  276 

chronic,  277 

diffuse,  277 

disseminated,  277 

syphilitic,  275 

transverse,  273 


Myelitis,  tubercular,  275 

Myelocele,  56 

Myoclonia,  61,  516 

Myoclonia  with  epilepsy,  518 

Myokymia,  518 

Myopathy,  321 

facio-scapulo-humeral  type,  323 
pseudo-hypertrophic  type,  322 
scapulo-humeral  type,  323 

Myosis,  246,  309 

Myospasm,  524 

Myotonia  atrophica,  329 
congenita,  525 

Myotonic  reaction,  526 

Myx oedema,  567 

strumipriva,  569 


Natatory  chorea,  468 
Neck  reflex,  177 
Negri's  bodies,  605 
Neosalvarsan,  350 
Nerve-cell,  49 

-fibers,  49 

-stretching,  434 
Neuralgia,  430 
Neurasthenia,  451 

sexual,  453 

symptomatica,  454 
Neurasthenoid,  362 
Neurectomy.  434 
Neuritic  atrophy,  325 
Neuritis,  377 

ascending,  381 

etiology,  380 

hypertrophic  interstitial,  383 

ischemic,  383 

multiple,  385 

occupation,  423 

pathology,  377 

periaxile,  379 

prognosis,  382 

segmentary,  379 

symptoms,  382 

treatment,  383 
Neurofibrils,  49 
Neuroglia,  50 
Neurolysis,  443 
Neuroma,  384,  516 
Neurones,  association,  51 

doctrine,  49 

motor,  8,  50,  320 

sensory,  8,  9,  51,  243 
Neuroparalytic  keratitis,  405 
Neuropathy,  456 
Neurorhexis,  434 
Neuroses  (occupation),  527 
Neurotropic  action,  351 
Night  terrors,  487 
Noguchi,  365 

Nonne's  Phase  I,  356,  358 
Nucleus,  accessory,  40 

caudate,  27 

cuneatus,  14 

dentate,  40 

of  fourth  nerve,  22 

gracilis,  13 


INDEX 


615 


Nucleus,  hypothalamic,  22 

lenticular,  29 

pontis,  19 

red,  21,  23 

of  third  nerve,  22 
Nystagmoid  tic,  508 
Nystagmus,  172,  215,  298 

acquired,  403 

congenital,  403 

horizontal  403 

miners',  404 

rotatory,  403 

vertical,  403 

vestibular,  404 

Obex,  15 
Obsessions,  457 
Obstetrical  palsy,  417 
Obturator  nerve,  426 

neuralgia,  444 
Occupation  neuritis,  423 

neuroses,  527 

spasms,  527 
Ocular  palsies,  402 
Oculo-motor  nerve,  401 

-pupillary  disturbances,  283,  417,  41^ 
(Edema  (of  brain),  145 

angioneurotic,  584 

hereditary,  585 

of  the  papilla,  172 
Olfactory  nerve,  399 
Olives,  12,  17 

Ophthalmic  herpes  zoster,  436,  448 
Ophthalmoplegia,  170 

progressive  nuclear,  224,  401 
Opisthotonos,  602 
Opotherapy,  565 
Oppenheim,  73,  91,  327,  505 
Optic  aphasia,  135 

apparatus,  20,  40 

atrophy,  215,  246,  298,  400,  573 

nerve,  400 

neuritis,  109,  147,  176,  243,  400,  573 

radiations,  32 

thalamus,  167 
Osteoma,  145 

Pachymeningitis,  171,  307,  331,  344,  355 

hemorrhagic,  111 
Palpebral  tic,  508 
Papilla,  217 

Paradoxical  reflex,  73,  91,  150 
Paresthesia,  68,  467 
Paragraphia,  131,  132 
Paralysis  agitans,  529 

acute  ascending,  396 

alternate,  151 

associative,  402 

Brown-Sequard's,  283 

complete,  61 

crossed,  151,  234,  237 

flaccid,  61,  90,  266,  398 

family  spastic,  257 

incomplete,  61 

motor,  153 

obstetrical,  120 


Paralysis,  periodic,  398 
spastic,  61,  90,  346 
spinal  (infantile),  264 
Paralysis  of  cranial  nerves,  399 
first  nerve,  399 
second,  400 
third,  401 
fourth,  401 
fifth,  405 
sixth,  401 
seventh,  406 
eighth,  411 
ninth,  412 
tenth,  412 
eleventh,  414 
twelfth,  415 
Paralysis  of  spinal  nerves,  415 
upper  cervical,  415 

phrenic,  415 
lower  cervical,  416 

brachial  plexus,  416 
lumbo-sacral,  425 
Paralytic  chorea,  497 
Paramyoclonus  multiplex,  517 
Paramyotonia  congenita,  526 
Paraplegia,  61,  117,  119,  132,  256,  258,  259 
Parasyphilitic  diseases,  252,  336 
Paresis,  pathology,  353 
diagnosis,  362 
etiology,  363 
prognosis,  362 
symptoms,  356 
treatment,  363 
Parkinson's  disease,  529 
Pathetic  nerve,  401 
Pathogenetic  link,  463 
Pavor  nocturnus,  487 
Peduncles,  cerebral,  19 

cerebellar,  19 
Peduncular  syndrome,  241 
Perforated  space  (posterior),  20 
Pellagra,  608 
Perineal  neuralgia,  447 
Periodic  paralysis,  398 
Peroneal  nerves,  426 

type  of  muscular  atrophy,  325 
Persuasion,  462,  480 
Petit  mal,  485 
Phobia,  457 
Photophobia,  187 
Phrenic  nerve,  415 
Pia  mater  of  brain,  45 

of  spinal  cord,  9,  10 
Pianist's  cramp,  528 
Pineal  body,  26 
Pithiatisme,  479 
Pituitary  body,  26,  154,  574 
Pleurodynia,  438 
Pneumogastric  nerve,  412 
Polioencephalitis,  104,  112 
inferior,  226 
superior,  223,  224 
Polioencephalomyelitis,  224 
Poliomyelitis  (acute),  264 
etiology,  265 
diagnosis,  269 


6i6 


INDEX 


Poliomyelitis,  pathogenesis,  265 

prognosis,  267 

symptoms,  266 

treatment,  270 
Polynucleosis,  372 
Pons,  16,  19,  236 
Ponto-cerebellar  angle,  217,  240 
Popliteal  nerves,  external,  427 

internal,  428 
Porencephaly,  52,  114 
Posterior  columns,  6 

cornua,  6 

longitudinal  bundle,  17,  22 

sclerosis,  242 
Postero-lateral  sclerosis,  259 
Potts'  disease,  307 
Preacher's  hand,  288,  332 
Precipitin  reaction,  181,  183,  195,  198 
Prefrontal  lobe,  81 
Primary  lateral  sclerosis,  256 
Progressive  muscular  atrophy,  314 
Projection  fibers,  31 
Propulsion.  60 
Protopathic,  70 
Pseudo-bulbar  palsy,  229 

-hypertrophy,  62,  322 

-meningitis,  181,  466 

-paresis,  363 

-ptosis,  467 

-tetanus,  522 

-tetany,  522 
Psychic  blindness,  133 
Psychanalysis,  462 
Psychasthenia,  456 
Psychoneuroses,  456 
Psychotherapeutics,  461,  480 
Ptosis,  246,  401,  402 
Puerperal  neuritis,  392 
Pulvinar,  24 
Pupils,  368 
Purkinje's  cells,  49 
Putamen.  30 
Pyramidal  bundle,  8 

crossed  tract,  8 

tracts,  8,  19,  35 
Pyramids,  8,  16 

Quadrigeminal  bodies,  20,  152,  169 
Quincke,  182,  584 

Rabies,  605 

Radiculalgia,  429 

Radicular  anaesthesia,  69,  287,  332 

sciatica,  429 
Radiculitis,  428 
Railroad  spine,  302 
Raynaud's  disease,  587 
Reactions  of  degeneration,  66,  267,  316,  332, 

4i7 
Recklinghausen's  disease,  384 
Recurrent  larnygeal  nerve,  413 
Red  nucleus,  21,  23 
Reflexes,  71 

abduction,  93 

Achilles',  71,  247 

adduction,  93 


Reflexes,  anal,  73 

ankle-clonus,  71,  90 

anticus,  94 

Babinski's,  73,  91,  94 

biceps,  72 

Chadwick's,  91 

Claude's,  94 

contralateral,  71 

cremasteric,  73 

Gordon's,  73,  91,  93 

Grasset's,  93 

hand,  93 

interossei,  92 

Marie's,  94 

masseter,  71 

neck,  177 

Oppenheim's,  73,  91 

patellar,  72,  247 

plantar,  73 

Schafer's,  91 

thumb,  93 

triceps,  71 

wrist,  93 
Reflex  neuralgia,  437 
Regeneration,  380 
Remissions,  366 
Restiform  bodies,  12,  14,  19 
Retropulsion,  60,  531 
Rhachischisis,  58 
Rhombencephalon,  11 
Risus  sardonicus,  602 
Rolando,  6 

Romberg's  sign,  59,  244 
Roots  of  spinal  nerves,  9,  243 
Rossolimo,  329 

Sacro-coxalgia,  441 

Saddle-shaped  anaesthesia,  292 

Salvarsan,  253,  350 

Sarcoma,  143 

Schlesinger's  sign,  521 

Schwab's  and  Allison's  operation,  122 

Sciatic  nerve,  426 

neuralgia,  439 
Scleroderma,  579 
Sclerosis  of  brain,  114 

combined,  259 

disseminated,  259 

insular,  295 

lateral,  256 

multiple,  295 

posterior,  242 

postero-lateral,  259 
Sclerosis,  263 
Self-suggestion,  469 
Senile  neuritis,  394 
Saltatory  chorea,  468 
Sensory  aphasia,  132 

dissociation,  69,  287 

neurones,  8 

phenomena,  68 
Septum,  3 

lucidum,  40 
Serotherapy,  194 
Serous  spinal  meningitis,  ^^$ 
Sexual  neurasthenia,  453 


INDEX 


617 


Sexual  trauma,  464 
Shaking  palsy,  529 
Shoemaker's  cramp,  528 
Shultze's  comma,  8 
Sicard,  254,  476 
Sight,  70 
Sinus  phlebitis,  in 

cavernous,  200 

longitudinal,  200 

transverse,  200 
Smell,  70,  151 
Softening  of  brain,  86,  97 

diagnosis,  96 

etiology,  87 

localization,  98 

pathology,  86 

prognosis,  96 

symptoms,  89 

treatment,  99 
Softening  of  cerebellum,  221 

medulla,  234 

pons,  238 
Somnambulism,  470 
Spasm,  facial,  514 

nutans,  511 
Spastic  paraplegia,  256 

spinal  paralysis,  346 
Spatapoplexie,  101 
Speech  scanning,  297 

spasmodic,  357 
Spermatic  neuralgia,  447 
Sphincters,  74,  274 
Spina  bifida,  55 
Spinal  accessory  nerve,  414 

anaesthesia,  443 

apoplexy,  280 

arteries,  n 

cord,  1,  242 

meningitis,  269,  329,  333 

nerves,  9,  415 

paralysis  (infantile),  264 

paralysis  (spastic),  346 

veins,  n 
Status  epilepticus,  484 
Stelwag's  sign,  560 
Steppage  gait,  386,  391,  427 
Stereognosis,  69,  79 
Stigmata,  465 
Stoffel's  operation,  123 
Strabismus,  400 
Striae  acusticse,  15 
Strumpell,  104 
Strychnin  poisoning,  603 
Stuttering,  469 
St.  Vitus  dance,  495 
Subarachnoid  space,  45 
Subdural  cavity,  10 
Suggestibility,  468 
Suggestion,  461,  469,  478,  480 
Sulcus,  3 

median,  13 
Supra-scapular  nerve,  419 
Sydenham's  chorea,  495 
Sylvius  aqueduct,  21 
Sympathetic  nervous  system,  560 
Syphilis,  117,  336,  365,  372 


Syphilis  of  brain,  337 

cerebro-spinal,  347,  364 

congenital,  365 

hereditary,  349 

of  peripheral  nerves,  348 

of  spinal  cord,  347 
Syphilitic  arteritis,  340 

hemiplegia,  341 

meningo-encephalitis,  250 

mental  disturbances,  348 
Syphiloma,  144 
Syringomyelia,  286 
Systematic  exercises,  255 

Tabes  dorsalis,  242 

juvenile,  256 
Tabetic  foot,  248 
Taches  cerebrales,  176 
Taenia  semicircularis,  24 
Taste,  71,  151 
Tegmentum,  19,  21 
Tela  chorioidea,  15,  38,  46 
Telegraphist's  cramp,  528 
Telencephalon,  26 
Temporal  lobe,  80 
Tender  spots,  431,  435,  437,  439 
Tenotomy,  271 
Tentorium  cerebelli,  44 
Testicular  neuralgia,  444 
Tetanus,  602 

cephalic,  605 

neonatorum,  603 
Tetany,  519 

gastric,  523 
Thalamus  opticus,  22,  24,  167 
Third  ventricle,  25 
Thomsen's  disease,  525 
Thoracic  nerve,  418 

segment,  1 
Thrombosis,  86,  88,  199,  237,  342 
Tic,  61,  507 

convulsif,  510 

douloureux,  436 

of  face,  508 

of  head,  509 

of  neck,  508 

laryngeal,  510 

nystagmoid,  508 

palpebral,  508 

psychic,  511 

Salaam,  511 
Tinnitus  aurium,  411 
Titubation,  60,  213,  2g8 
Torticollis,  mental,  509 

spasmodic,  509 
Tracts,  7 

antero-lateral,  9 

direct  pyramidal,  8 

Flechsig's,  8 

Gower's,  8 

Loewenthal's,  9 
Transcortical  aphasia,  134 
Traumatic  lesions  of  cord,  302 

neuroses,  548 

psychoses,  555 
Tremor,  61 


6i8 


INDEX 


Tremor,  intention,  262,  297 

passive,  530 
Trigeminal  nerve,  405 
Trigonum,  acusticum,  15 
habenulae,  15 
hypoglossi,  15 
Trismus,  602 
Trophoneuroses,  560 
Trousseau's  sign,  521 
Tuberculoma,  144 
Tumors  of  brain,  143 
course,  155 
diagnosis,  156 
etiology,  158 
pathogenesis,  154 
pathology,  143 
symptoms,  146 
treatment,  158 
of  cerebellum,  213 
cord,  306 
pons,  238 
Turck's  bundle,  8 

Ulnar  nerve,  422 
Uncinate  fits,  151 
Unverricht's  myoclonia,  518 

Velum,  anterior  medullary,  15 
posterior  medullary,  15 

Ventricles,  fifth,  40 
fourth,  15 
lateral,  38 


Ventricles,  third,  25 
Verbal  amnesia,  135 
Vertebral  cancer,  311 
Vertigo,  109,  147,  263,  411,  544 
Vestibular  nerve,  411 
Violinist's  cramp,  528 
Visual  apparatus,  40 

disturbances,  467 
Vomiting,  147 
Von  Graefe's  sign,  560 

Wasserman's  test,  117,  126,351,356,358,365, 

373,  374 
Weber's  syndrome,  239 
Wechselbaum's  meningococcus,  372 
Weir-Mitchell,  585 
Wernicke's  aphasia,  78,  130,  133 

area,  78,  137 

polio-encephalitis,  superior,  104 

pupil,  141 
Westphal's  sign,  247 
Wet  brain,  597 
White  substance  of  brain,  31 

of  spinal  cord,  6 
Word-blindness,  78,  133,  150 

-deafness,  78,  132,  151 
Wrist-drop,  386,  421 
Writer's  cramp,  527 

Zona,  447 

Zoster,  herpes,  436,  447 


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This  book  is  due  on  the  date  indicated  below,  or  at  the 
expiration  of  a  definite  period  after  the  date  of  borrowing,  as 
provided  by  the  library  rules  or  by  special  arrangement  with 
the  Librarian  in  charge. 

DATE  BORROWED 

DATE  DUE 

DATE  BORROWED 

DATE  DUE 

| 

- 

CD  c  1    1340 

C28  (747)  MIOO 

** 


